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Disease on EC 3.1.4.12 - sphingomyelin phosphodiesterase and Organism(s) Homo sapiens and UniProt Accession Q9NXE4

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Aberrant Crypt Foci
Ursolic acid inhibits the formation of aberrant crypt foci and affects colonic sphingomyelin hydrolyzing enzymes in azoxymethane-treated rats.
Abortion, Spontaneous
Niemann-Pick disease: prenatal diagnoses and studies of sphingomyelinase activities.
Abscess
Clostridium septicum myonecrosis presenting as a parapharyngeal abscess in a patient with aplastic anemia.
Effect immunization with highly purified alpha- and beta-toxins on staphylococcal mastitis in rabbits.
Acne Vulgaris
Propionibacterium acnes CAMP factor and host acid sphingomyelinase contribute to bacterial virulence: potential targets for inflammatory acne treatment.
Acute Coronary Syndrome
Elevation of ceramide and activation of secretory acid sphingomyelinase in patients with acute coronary syndromes.
Acute Generalized Exanthematous Pustulosis
Acute generalized exanthematous pustulosis and Coombs-positive hemolytic anemia in a child following Loxosceles reclusa envenomation.
Acute Kidney Injury
Altered sphingomyelinase and ceramide expression in the setting of ischemic and nephrotoxic acute renal failure.
Acute Lung Injury
A novel visible range FRET probe for monitoring acid sphingomyelinase activity in living cells.
Acid sphingomyelinase mediates murine acute lung injury following transfusion of aged platelets.
IKK NBD peptide inhibits LPS induced pulmonary inflammation and alters sphingolipid metabolism in a murine model.
PAF-mediated pulmonary edema: a new role for acid sphingomyelinase and ceramide.
Platelet-activating factor reduces endothelial NO production - role of acid sphingomyelinase.
Adenocarcinoma
'Patchiness' and basic cancer research: unravelling the proteases.
Detection of alkaline sphingomyelinase activity in human stool: proposed role as a new diagnostic and prognostic marker of colorectal cancer.
Familial adenomatous polyposis is associated with a marked decrease in alkaline sphingomyelinase activity: a key factor to the unrestrained cell proliferation?
Reciprocal relationship between cytosolic NADH and ENOX2 inhibition triggers sphingolipid-induced apoptosis in HeLa cells.
[Synchronous Multiple Primary Lung Cancer Dignosed ?by Different Phenotype-genotype: A Case Report and Literature Review].
Adenocarcinoma of Lung
Synchronous Bilateral Lung Cancer With Discordant Histology.
Adenoma
Chronic colitis is associated with a reduction of mucosal alkaline sphingomyelinase activity.
Effects of ursodeoxycholate and other bile salts on levels of rat intestinal alkaline sphingomyelinase: a potential implication in tumorigenesis.
Familial adenomatous polyposis is associated with a marked decrease in alkaline sphingomyelinase activity: a key factor to the unrestrained cell proliferation?
Reduction in alkaline sphingomyelinase in colorectal tumorigenesis is not related to the APC gene mutation.
Adenomatous Polyposis Coli
Familial adenomatous polyposis is associated with a marked decrease in alkaline sphingomyelinase activity: a key factor to the unrestrained cell proliferation?
Reduction in alkaline sphingomyelinase in colorectal tumorigenesis is not related to the APC gene mutation.
Aggressive Periodontitis
Sphingomyelin Phosphodiesterase 3 Enhances Cytodifferentiation of Periodontal Ligament Cells.
Alveolar Bone Loss
Acid sphingomyelinase deficiency exacerbates LPS-induced experimental periodontitis.
Alzheimer Disease
Acid sphingomyelinase modulates the autophagic process by controlling lysosomal biogenesis in Alzheimer's disease.
Cambinol, a novel inhibitor of neutral sphingomyelinase 2 shows neuroprotective properties.
Fibrillar amyloid-beta peptides kill human primary neurons via NADPH oxidase-mediated activation of neutral sphingomyelinase. Implications for Alzheimer's disease.
Fibrillar amyloid-beta-activated human astroglia kill primary human neurons via neutral sphingomyelinase: implications for Alzheimer's disease.
Interactions between neural membrane glycerophospholipid and sphingolipid mediators: a recipe for neural cell survival or suicide.
Neutral Sphingomyelinase-2 Deficiency Ameliorates Alzheimer's Disease Pathology and Improves Cognition in the 5XFAD Mouse.
Novel Human Neutral Sphingomyelinase 2 Inhibitors as Potential Therapeutics for Alzheimer's Disease.
Role of sphingomyelinases in neurological disorders.
Roles and regulation of neutral sphingomyelinase-2 in cellular and pathological processes.
The Roles of Neutral Sphingomyelinases in Neurological Pathologies.
Tunable regulatory activities of 1,10-phenanthroline derivatives towards acid sphingomyelinase and Zn(ii)-amyloid-?.
[Advance in research on regulatory mechanism and functions of neutral sphingomyelinse 2].
Anaphylaxis
Mechanistic characterization and inhibition of sphingomyelinase C over substituted Iron Schiff bases of chitosan adsorbed on glassy carbon electrode.
Anaplasmosis
Functional inhibition or genetic deletion of acid sphingomyelinase bacteriostatically inhibits Anaplasma phagocytophilum infection in vivo.
Anemia
Acute Gaucher Disease-Like Condition in an Indian Infant with a Novel Biallelic Mutation in the Prosaposin Gene.
Functional consequences of sphingomyelinase-induced changes in erythrocyte membrane structure.
Liver cell death and anemia in Wilson disease involve acid sphingomyelinase and ceramide.
Susceptibility to staphylococcal alpha-toxin of Friend virus-infected murine erythroblasts during differentiation.
Anemia, Hemolytic
Clostridium Perfringens Bacteremia with Acute Hemolytic Anemia in the Setting of Endometrial Malignancy.
Anemia, Sickle Cell
Acid sphingomyelinase is activated in sickle cell erythrocytes and contributes to inflammatory microparticle generation in SCD.
Angiokeratoma
A novel association between angiokeratoma corporis diffusum and acid sphingomyelinase deficiency.
Anthrax
Membrane insertion: The strategies of toxins (review).
Arthritis
Regulation of Arthritis Severity by the Acid Sphingomyelinase.
Severity of group B streptococcal arthritis is correlated with beta-hemolysin expression.
Arthritis, Infectious
Alpha-toxin and gamma-toxin jointly promote Staphylococcus aureus virulence in murine septic arthritis.
Arthritis, Rheumatoid
Acid sphingomyelinase activity is elevated in the serum of rheumatoid arthritis patients, suppressed by anti-TNF-? treatment.
Acid Sphingomyelinase and Acid ?-Glucosidase 1 Exert Opposite Effects on Interleukin-1?-Induced Interleukin 6 Production in Rheumatoid Arthritis Fibroblast-Like Synoviocytes.
Regulation of Arthritis Severity by the Acid Sphingomyelinase.
Secretory sphingomyelinase (S-SMase) activity is elevated in patients with rheumatoid arthritis.
Arthrogryposis
Loss of SMPD4 Causes a Developmental Disorder Characterized by Microcephaly and Congenital Arthrogryposis.
Arthus Reaction
Different pathways leading to cutaneous leukocytoclastic vasculitis in mice.
Asthma
Acid sphingomyelinase regulates TH 2-cytokine release and bronchial asthma.
Ceramide induction of COX-2 and PGE(2) in pulmonary A549 cells does not involve activation of NF-kappaB.
Immunoregulatory role of acid sphingomyelinase in allergic asthma.
Sphingomyelin metabolism in erythrocyte membrane in asthma.
Astrocytoma
Cytokine and intracellular signaling regulation of tissue factor expression in astrocytes.
Stimulation of the sphingomyelin pathway induces interleukin-6 gene expression in human astrocytoma cells.
Ataxia
Disruption of myelin sheaths in mouse brain in vitro and in vivo by staphylococcal alpha-toxin.
Highly variable neural involvement in sphingomyelinase-deficient Niemann-Pick disease caused by an ancestral Gypsy mutation.
Atherosclerosis
A high-throughput sphingomyelinase assay using natural substrate.
A novel high-throughput screening format to identify inhibitors of secreted acid sphingomyelinase.
A Robust Liposomal Platform for Direct Colorimetric Detection of Sphingomyelinase Enzyme and Inhibitors.
Acid sphingomyelinase promotes lipoprotein retention within early atheromata and accelerates lesion progression.
Acid sphingomyelinase, autophagy, and atherosclerosis.
Acute systemic inflammation up-regulates secretory sphingomyelinase in vivo: a possible link between inflammatory cytokines and atherogenesis.
Alkaline sphingomyelinase: an old enzyme with novel implications.
Amitriptyline inhibits nonalcoholic steatohepatitis and atherosclerosis induced by high-fat diet and LPS through modulation of sphingolipid metabolism.
Boswellic acid inhibits expression of acid sphingomyelinase in intestinal cells.
Ceramide in lipid particles enhances heparan sulfate proteoglycan and low density lipoprotein receptor-related protein-mediated uptake by macrophages.
Contribution of acid sphingomyelinase to angiotensin II-induced vascular adventitial remodeling via membrane rafts/Nox2 signal pathway.
Control of autophagy maturation by acid sphingomyelinase in mouse coronary arterial smooth muscle cells: protective role in atherosclerosis.
Dual effect of ceramide on human endothelial cells: induction of oxidative stress and transcriptional upregulation of endothelial nitric oxide synthase.
Effect of dietary cholesterol and high fat on ceramide concentration in rat tissues.
Elevation of ceramide and activation of secretory acid sphingomyelinase in patients with acute coronary syndromes.
Facile Method for Specifically Sensing Sphingomyelinase in Cells and Human Urine Based on a Ratiometric Fluorescent Nanoliposome Probe.
Human vascular endothelial cells are a rich and regulatable source of secretory sphingomyelinase. Implications for early atherogenesis and ceramide-mediated cell signaling.
Interactions between sphingomyelin and cholesterol in low density lipoproteins and model membranes.
Modification of LDL with human secretory phospholipase A(2) or sphingomyelinase promotes its arachidonic acid-releasing propensity.
Modulation of sphingomyelinase-induced cholesterol esterification in fibroblasts, CaCo2 cells, macrophages and smooth muscle cells.
Neutral sphingomyelinase: past, present and future.
Neutral/alkaline and acid ceramidase activities are actively released by murine endothelial cells.
nSMase2 (Type 2-Neutral Sphingomyelinase) Deficiency or Inhibition by GW4869 Reduces Inflammation and Atherosclerosis in Apoe-/- Mice.
Oxidized phospholipids in minimally modified low density lipoprotein induce apoptotic signaling via activation of acid sphingomyelinase in arterial smooth muscle cells.
Platelet arachidonic acid metabolism in severe cerebrovascular disease.
Rabbit aorta and human atherosclerotic lesions hydrolyze the sphingomyelin of retained low-density lipoprotein. Proposed role for arterial-wall sphingomyelinase in subendothelial retention and aggregation of atherogenic lipoproteins.
Secretory sphingomyelinase, a product of the acid sphingomyelinase gene, can hydrolyze atherogenic lipoproteins at neutral pH. Implications for atherosclerotic lesion development.
Secretory sphingomyelinase.
Short-term magnesium deficiency downregulates telomerase, upregulates neutral sphingomyelinase and induces oxidative DNA damage in cardiovascular tissues: relevance to atherogenesis, cardiovascular diseases and aging.
Sphingolipids in atherosclerosis and vascular biology.
Sphingomyelin phosphodiesterase acid-like 3A (SMPDL3A) is a novel nucleotide phosphodiesterase regulated by cholesterol in human macrophages.
Sphingomyelinase activity of LDL: a link between atherosclerosis, ceramide, and apoptosis?
Sphingomyelinase converts lipoproteins from apolipoprotein E knockout mice into potent inducers of macrophage foam cell formation.
Sphingomyelinase induces aggregation and fusion of small very low-density lipoprotein and intermediate-density lipoprotein particles and increases their retention to human arterial proteoglycans.
Sphingomyelinase, an enzyme implicated in atherogenesis, is present in atherosclerotic lesions and binds to specific components of the subendothelial extracellular matrix.
Sphingomyelinases: their regulation and roles in cardiovascular pathophysiology.
Strong Inhibition of Secretory Sphingomyelinase by Catechins, Particularly by (-)-Epicatechin 3-O-Gallate and (-)-3'-O-Methylepigallocatechin 3-O-Gallate.
Suppressing the intestinal farnesoid X receptor/sphingomyelin phosphodiesterase 3 axis decreases atherosclerosis.
The cellular trafficking and zinc dependence of secretory and lysosomal sphingomyelinase, two products of the acid sphingomyelinase gene.
The Polycomb group protein EED couples TNF receptor 1 to neutral sphingomyelinase.
The Roles of Neutral Sphingomyelinases in Neurological Pathologies.
Autoimmune Diseases
Molecular modeling of human neutral sphingomyelinase provides insight into its molecular interactions.
Bacteremia
Characterization of alpha-toxin hla gene variants, alpha-toxin expression levels, and levels of antibody to alpha-toxin in hemodialysis and postsurgical patients with Staphylococcus aureus bacteremia.
Distinguishing complicated from uncomplicated bacteremia caused by Staphylococcus aureus: the value of "new" and "old" serological tests.
Evaluation of seven different enzyme-linked immunosorbent assays for serodiagnosis of Staphylococcus aureus bacteremia.
Hemolysin and K antigens in relation to serotype and hemagglutination type of Escherichia coli isolated from extraintestinal infections.
Isogenic group B streptococci devoid of capsular polysaccharide or beta-hemolysin: pulmonary hemodynamic and gas exchange effects during bacteremia in piglets.
Multimechanistic Monoclonal Antibodies (MAbs) Targeting Staphylococcus aureus Alpha-Toxin and Clumping Factor A: Activity and Efficacy Comparisons of a MAb Combination and an Engineered Bispecific Antibody Approach.
Serology of Staphylococcus aureus infections using multiple antigens and serial serum samples.
Bacterial Infections
Defective Acid Sphingomyelinase Pathway with Pseudomonas aeruginosa Infection in Cystic Fibrosis.
Extravasation of staphylococcal alpha-toxin in normal and injured CNS regions lacking blood-brain barrier function: observations after ventral root replantation.
Neutral sphingomyelinase 2 is a key factor for PorB-dependent invasion of Neisseria gonorrhoeae.
The role of acid sphingomyelinase and modulation of sphingolipid metabolism in bacterial infection.
Beckwith-Wiedemann Syndrome
Acid sphingomyelinase deficiency in Beckwith Wiedemann syndrome.
Bone Resorption
Sphingomyelinase and ceramide inhibit formation of F-actin ring in and bone resorption by rabbit mature osteoclasts.
Botulism
Investigation of Clostridium botulinum group III's mobilome content.
Brain Abscess
Diminished virulence of an alpha-toxin mutant of Staphylococcus aureus in experimental brain abscesses.
Brain Concussion
The Role of Acid Sphingomyelinase Inhibition in Repetitive Mild Traumatic Brain Injury.
Brain Injuries
Acid sphingomyelinase deficiency protects mitochondria and improves function recovery after brain injury.
Inhibition of extracellular vesicle pathway using neutral sphingomyelinase inhibitors as a neuroprotective treatment for brain injury.
Sphingomyelinase and ceramide analogs induce vasoconstriction and leukocyte-endothelial interactions in cerebral venules in the intact rat brain: Insight into mechanisms and possible relation to brain injury and stroke.
Brain Injuries, Traumatic
Acid Sphingomyelinase Inhibition Mitigates Histopathologic and Behavioral Changes in a Murine Model of Traumatic Brain Injury.
Neutral Sphingomyelinase Inhibition Alleviates LPS-Induced Microglia Activation and Neuroinflammation after Experimental Traumatic Brain Injury.
Brain Ischemia
Homozygous Smpd1 deficiency aggravates brain ischemia/ reperfusion injury by mechanisms involving polymorphonuclear neutrophils, whereas heterozygous Smpd1 deficiency protects against mild focal cerebral ischemia.
Pivotal role for acidic sphingomyelinase in cerebral ischemia-induced ceramide and cytokine production, and neuronal apoptosis.
Role of sphingomyelinases in neurological disorders.
The Acid Sphingomyelinase/ Ceramide System as Target for Ischemic Stroke Therapies.
Tumor necrosis factor alpha increases neuronal vulnerability to excitotoxic necrosis by inducing expression of the AMPA-glutamate receptor subunit GluR1 via an acid sphingomyelinase- and NF-kappaB-dependent mechanism.
Upregulation of ceramide and its regulating mechanism in a rat model of chronic cerebral ischemia.
[Synthesis of sphingomyelin analogues as a sphingomyelinase inhibitor and the application as a blocking agent of extracellular stress signaling]
Brain Neoplasms
Sphingolipid Metabolism in Glioblastoma and Metastatic Brain Tumors: A Review of Sphingomyelinases and Sphingosine-1-Phosphate.
Breast Neoplasms
'Patchiness' and basic cancer research: unravelling the proteases.
A novel role for protein kinase Cdelta-mediated phosphorylation of acid sphingomyelinase in UV light-induced mitochondrial injury.
alpha-TEA-induced death receptor dependent apoptosis involves activation of acid sphingomyelinase and elevated ceramide enriched cell surface membranes.
Association between CLN3 (Neuronal Ceroid Lipofuscinosis, CLN3 Type) Gene Expression and Clinical Characteristics of Breast Cancer Patients.
Comparative epigenomics of human and mouse mammary tumors.
Disruption of retinoic acid receptor alpha reveals the growth promoter face of retinoic acid.
Glutathione regulation of neutral sphingomyelinase in tumor necrosis factor-alpha-induced cell death.
Molecular characterization of human breast tumor vascular cells.
Neutral Sphingomyelinase 2 Heightens Anti-Melanoma Immune Responses and Anti-PD-1 Therapy Efficacy.
Neutral sphingomyelinase and breast cancer research.
Novel quinuclidinone derivative 8a induced apoptosis in human MCF-7 breast cancer cell lines.
Quinuclidinone derivative 6 induced apoptosis in human breast cancer cells via sphingomyelinase and JNK signaling.
Regulated secretion of acid sphingomyelinase: implications for selectivity of ceramide formation.
Role for mammalian neutral sphingomyelinase 2 in confluence-induced growth arrest of MCF7 cells.
Selective hydrolysis of a mitochondrial pool of sphingomyelin induces apoptosis.
Transcriptional regulation of neutral sphingomyelinase 2 gene expression of a human breast cancer cell line, MCF-7, induced by the anti-cancer drug, daunorubicin.
Transcriptional regulation of neutral sphingomyelinase 2 in all-trans retinoic acid-treated human breast cancer cell line, MCF-7.
Bronchiolitis
Elevation of Serum Acid Sphingomyelinase Activity in Children with Acute Respiratory Syncytial Virus Bronchiolitis.
Carcinogenesis
Alkaline sphingomyelinase (NPP7) in hepatobiliary diseases: A field that needs to be closely studied.
Cloning of alkaline sphingomyelinase from rat intestinal mucosa and adjusting of the hypothetical protein XP_221184 in GenBank.
Dietary sphingomyelin inhibits colonic tumorigenesis with an up-regulation of alkaline sphingomyelinase expression in ICR mice.
Effects of phospholipids on sphingomyelin hydrolysis induced by intestinal alkaline sphingomyelinase: an in vitro study.
Effects of ursodeoxycholate and other bile salts on levels of rat intestinal alkaline sphingomyelinase: a potential implication in tumorigenesis.
Enhanced colonic tumorigenesis in alkaline sphingomyelinase (NPP7) knockout mice.
Identification of aberrant forms of alkaline sphingomyelinase (NPP7) associated with human liver tumorigenesis.
Mutations in the neutral sphingomyelinase gene SMPD3 implicate the ceramide pathway in human leukemias.
Neutral sphingomyelinase-3 is a DNA damage and nongenotoxic stress-regulated gene that is deregulated in human malignancies.
Next Generation Sequencing Reveals a Synchronous Trilateral Lung Adenocarcinoma Case with Distinct Driver Alterations of EGFR 19 Deletion or EGFR 20 Insertion or EZR-ROS1 Fusion.
Pancreatic and mucosal enzymes in choline phospholipid digestion.
Purification, characterization, and expression of rat intestinal alkaline sphingomyelinase.
Reduction in alkaline sphingomyelinase in colorectal tumorigenesis is not related to the APC gene mutation.
Understanding the molecular activity of alkaline sphingomyelinase (NPP7) by computer modeling.
Carcinoma
Abnormal methylation status of FBXW10 and SMPD3, and associations with clinical characteristics in clear cell renal cell carcinoma.
Acid sphingomyelinase activity as an indicator of the cell stress in HPV-positive and HPV-negative head and neck squamous cell carcinoma.
Acidic sphingomyelinase induced by electrophiles promotes proinflammatory cytokine production in human bladder carcinoma ECV-304 cells.
Alkaline sphingomyelinase activity is decreased in human colorectal carcinoma.
c-Abl Is an Upstream Regulator of Acid Sphingomyelinase in Apoptosis Induced by Inhibition of Integrins ?v?3 and ?v?5.
Different apoptosis ratios and gene expressions in two human cell lines after sevoflurane anaesthesia.
Effects of ursodeoxycholate and other bile salts on levels of rat intestinal alkaline sphingomyelinase: a potential implication in tumorigenesis.
Enhanced acid sphingomyelinase activity drives immune evasion and tumor growth in non-small cell lung carcinoma.
Glutathione regulation of neutral sphingomyelinase in tumor necrosis factor-alpha-induced cell death.
Increased liver tumor formation in neutral sphingomyelinase-2 deficient mice.
Lack of ceramide generation and altered sphingolipid composition are associated with drug resistance in human ovarian carcinoma cells.
Nrf2 up-regulates the induction of acidic sphingomyelinase by electrophiles.
Purified intestinal alkaline sphingomyelinase inhibits proliferation without inducing apoptosis in HT-29 colon carcinoma cells.
Sphingomyelin phosphodiesterase 3 methylation and silencing in oral squamous cell carcinoma results in increased migration and invasion and altered stress response.
Studies of the Biologic Activity of Purified Staphylococcal Alpha-Toxin: II. The Effect of Alpha-Toxin on Ehrlich Ascites Carcinoma Cells.
Synchronous Bilateral Lung Cancer With Discordant Histology.
TSAP6 is a novel candidate marker of poor survival in metastatic high-grade serous carcinoma.
[Synchronous Multiple Primary Lung Cancer Dignosed ?by Different Phenotype-genotype: A Case Report and Literature Review].
Carcinoma, Hepatocellular
A molecular basis for insulin resistance. Elevated serine/threonine phosphorylation of IRS-1 and IRS-2 inhibits their binding to the juxtamembrane region of the insulin receptor and impairs their ability to undergo insulin-induced tyrosine phosphorylation.
Celecoxib-mediated activation of endoplasmic reticulum stress induces de novo ceramide biosynthesis and apoptosis in hepatoma HepG2 cells mobilization.
Common inhibitory serine sites phosphorylated by IRS-1 kinases, triggered by insulin and inducers of insulin resistance.
Existence of Mg2+-dependent, neutral sphingomyelinase in nuclei of rat ascites hepatoma cells.
Exosomal neutral sphingomyelinase 1 suppresses hepatocellular carcinoma via decreasing the ratio of sphingomyelin/ceramide.
Human alkaline ceramidase 2 promotes the growth, invasion, and migration of hepatocellular carcinoma cells via sphingomyelin phosphodiesterase acid-like 3B.
Nuclear localization of neutral sphingomyelinase 1: biochemical and immunocytochemical analyses.
Plasma membrane sphingomyelin and the regulation of HMG-CoA reductase activity and cholesterol biosynthesis in cell cultures.
Regulation of UDP-glucose:ceramide glucosyltransferase-1 by ceramide.
Sphingomyelinase and ceramide suppress insulin-induced tyrosine phosphorylation of the insulin receptor substrate-1.
Sphingomyelinases and Liver Diseases.
Carcinoma, Non-Small-Cell Lung
Abnormal methylation of seven genes and their associations with clinical characteristics in early stage non-small cell lung cancer.
Ceramide mediates nanovesicle shedding and cell death in response to phosphatidylinositol ether lipid analogs and perifosine.
Enhanced acid sphingomyelinase activity drives immune evasion and tumor growth in non-small cell lung carcinoma.
Carcinoma, Renal Cell
Abnormal methylation status of FBXW10 and SMPD3, and associations with clinical characteristics in clear cell renal cell carcinoma.
Data on cell survival, apoptosis, ceramide metabolism and oxidative stress in A-494 renal cell carcinoma cell line treated with hesperetin and hesperetin-7-O-acetate.
Carcinoma, Squamous Cell
Acid sphingomyelinase activity as an indicator of the cell stress in HPV-positive and HPV-negative head and neck squamous cell carcinoma.
Radioresistance of human carcinoma cells is correlated to a defect in raft membrane clustering.
Cardiomyopathies
Adjustment of Dysregulated Ceramide Metabolism in a Murine Model of Sepsis-Induced Cardiac Dysfunction.
HILIC-MS-based metabolomics reveal that Astragalus polysaccharide alleviates doxorubicin-induced cardiomyopathy by regulating sphingolipid and glycerophospholipid homeostasis.
Cardiovascular Diseases
Acid sphingomyelinase downregulation alleviates vascular endothelial leptin resistance in rats.
Hemolytic and sphingomyelinase activities of Clostridium perfringens alpha-toxin are dependent on a domain homologous to that of an enzyme from the human arachidonic acid pathway.
Identification and biochemical characterization of an acid sphingomyelinase-like protein from the bacterial plant pathogen Ralstonia solanacearum that hydrolyzes ATP to AMP but not sphingomyelin to ceramide.
Short-term magnesium deficiency downregulates telomerase, upregulates neutral sphingomyelinase and induces oxidative DNA damage in cardiovascular tissues: relevance to atherogenesis, cardiovascular diseases and aging.
Sphingomyelinases: their regulation and roles in cardiovascular pathophysiology.
Cataplexy
Gelastic cataplexy in Niemann-Pick disease group C and related variants without generalized sphingomyelinase deficiency.
ceramidase deficiency
Acid Sphingomyelinase Deficiency Ameliorates Farber Disease.
Cerebral Infarction
Inhibition of sphingomyelinase activity helps to prevent neuron death caused by ischemic stress.
Chagas Disease
Trypanosoma cruzi modulates gene expression of plasma membrane repair-related proteins.
Chemical and Drug Induced Liver Injury
Activity of sphingomyelinase in rat liver in acute and chronic toxic hepatitis: proportion between peroxidative and phospholipase pathways of lipid bilayer modification.
Functional activity of sphingomyelin cycle in rat liver in chronic toxic hepatitis.
Chickenpox
Comparative study of immune status to infectious agents in elderly patients with multiple myeloma, Waldenstrom's macroglobulinemia, and monoclonal gammopathy of undetermined significance.
Relationship between endotoxin core, staphylococcal and varicella antibody levels and outcome following aortic valve replacement surgery: a prospective observational study.
Cholera
A simple method for the determination of the pore radius of ion channels in planar lipid bilayer membranes.
Cholera toxin B-subunit prevents activation and proliferation of human CD4+ T cells by activation of a neutral sphingomyelinase in lipid rafts.
Different types of cell death induced by enterotoxins.
Interleukin-1 beta stimulates sphingomyelin hydrolysis in cultured granulosa cells: evidence for a regulatory role of ceramide on progesterone and prostaglandin biosynthesis.
Melanogenesis in murine B16 cells exposed to Aeromonas hydrophila cytotoxic enterotoxin.
Microbial sphingomyelinase induces RhoA-mediated reorganization of the apical brush border membrane and is protective against invasion.
The role of bacterial and non-bacterial toxins in the induction of changes in membrane transport: implications for diarrhea.
[Antibacterial and anti-hemolysin activities of tea catechins and their structural relatives]
[Relationship between the anti-hemolysin activity and the structure of catechins and theaflavins]
Cholestasis
[Relationship of tumor necrosis factor alpha expression with activation of sphingomyelinase and lipid peroxidation after removal of cholestatic factor]
Cholestasis, Intrahepatic
Lithocholic acid disrupts phospholipid and sphingolipid homeostasis leading to cholestasis in mice.
Clostridium Infections
Role of alpha-toxin in Clostridium perfringens infection determined by using recombinants of C. perfringens and Bacillus subtilis.
Coinfection
Candida albicans Augments Staphylococcus aureus Virulence by Engaging the Staphylococcal agr Quorum Sensing System.
Linezolid Attenuates Lethal Lung Damage during Postinfluenza Methicillin-Resistant Staphylococcus aureus Pneumonia.
Colitis
Acid sphingomyelinase inhibition suppresses lipopolysaccharide-mediated release of inflammatory cytokines from macrophages and protects against disease pathology in dextran sulphate sodium-induced colitis in mice.
Chronic colitis is associated with a reduction of mucosal alkaline sphingomyelinase activity.
Deficiency of alkaline SMase enhances dextran sulfate sodium-induced colitis in mice with upregulation of autotaxin.
Expression of Alkaline Sphingomyelinase in Yeast Cells and Anti-inflammatory Effects of the Expressed Enzyme in a Rat Colitis Model.
Inhibition of lipopolysaccharide-induced release of interleukin-8 from intestinal epithelial cells by SMA, a novel inhibitor of sphingomyelinase and its therapeutic effect on dextran sulphate sodium-induced colitis in mice.
Intestinal Acid Sphingomyelinase Protects From Severe Pathogen-Driven Colitis.
VSL#3 probiotic upregulates intestinal mucosal alkaline sphingomyelinase and reduces inflammation.
Colitis, Ulcerative
VSL#3 probiotic upregulates intestinal mucosal alkaline sphingomyelinase and reduces inflammation.
Colitis-Associated Neoplasms
Deficiency of alkaline SMase enhances dextran sulfate sodium-induced colitis in mice with upregulation of autotaxin.
Colonic Neoplasms
Alkaline sphingomyelinase: an old enzyme with novel implications.
Cisplatin-induced CD95 redistribution into membrane lipid rafts of HT29 human colon cancer cells.
Curcumin decreases acid sphingomyelinase activity in colon cancer Caco-2 cells.
Dietary sphingomyelin inhibits colonic tumorigenesis with an up-regulation of alkaline sphingomyelinase expression in ICR mice.
Identification of one exon deletion of intestinal alkaline sphingomyelinase in colon cancer HT-29 cells and a differentiation-related expression of the wild-type enzyme in Caco-2 cells.
Inhibitory Effect of Lactobacillus plantarum Extracts on HT-29 Colon Cancer Cell Apoptosis Induced by Staphylococcus aureus and Its Alpha-Toxin.
Intestinal alkaline sphingomyelinase hydrolyses and inactivates platelet-activating factor by a phospholipase C activity.
Liver acid sphingomyelinase inhibits growth of metastatic colon cancer.
Purification, localization, and expression of human intestinal alkaline sphingomyelinase.
Purified intestinal alkaline sphingomyelinase inhibits proliferation without inducing apoptosis in HT-29 colon carcinoma cells.
Sphingosine Kinase Isoforms Regulate Oxaliplatin Sensitivity of Human Colon Cancer Cells through Ceramide Accumulation and Akt Activation.
Ursodeoxycholic acid differentially affects three types of sphingomyelinase in human colon cancer Caco 2 cells.
Ursodeoxycholic acid increases the activities of alkaline sphingomyelinase and caspase-3 in the rat colon.
Ursolic Acid Inhibits Acid Sphingomyelinase in Intestinal Cells.
Colorectal Neoplasms
Alkaline sphingomyelinase activity is decreased in human colorectal carcinoma.
Chronic colitis is associated with a reduction of mucosal alkaline sphingomyelinase activity.
Comparative lipidomics of 5-Fluorouracil-sensitive and -resistant colorectal cancer cells reveals altered sphingomyelin and ceramide controlled by acid sphingomyelinase (SMPD1).
Detection of alkaline sphingomyelinase activity in human stool: proposed role as a new diagnostic and prognostic marker of colorectal cancer.
Reduction in alkaline sphingomyelinase in colorectal tumorigenesis is not related to the APC gene mutation.
The Effects of ASMase Mediated Endothelial Cell Apoptosis in Multiple Hypofractionated Irradiations in CT26 Tumor Bearing Mice.
[Effect of jianpi-jiedu formula on tumor angiogenesis-relevant genes expression in colorectal cancer].
Communicable Diseases
Inhibitory effects of various plant polyphenols on the toxicity of Staphylococcal alpha-toxin.
Novel inhibitor of bacterial sphingomyelinase, SMY-540, developed based on three-dimensional structure analysis.
Pore-forming activity of alpha-toxin is essential for clostridium septicum-mediated myonecrosis.
Role of sphingomyelinase in infectious diseases caused by Bacillus cereus.
Selective killing of human monocytes and cytokine release provoked by sphingomyelinase (beta-toxin) of Staphylococcus aureus.
Stimulation of PAF-synthesis in pulmonary artery endothelial cells by Staphylococcus aureus alpha-toxin.
Congenital Abnormalities
Role of SMPD3 during Bone Fracture Healing and Regulation of Its Expression.
Smpd3 Expression in both Chondrocytes and Osteoblasts Is Required for Normal Endochondral Bone Development.
Sphingolipid metabolism and its role in the skeletal tissues.
Corneal Diseases
Effects of toxin production in a murine model of Staphylococcus aureus keratitis.
Corneal Edema
Histopathological studies of staphylococcal alpha-toxin: effects on rabbit corneas.
Corneal Injuries
Chemical Inhibition of Alpha-Toxin, a Key Corneal Virulence Factor of Staphylococcus aureus.
Effectiveness of Alpha-toxin Fab Monoclonal Antibody Therapy in Limiting the Pathology of Staphylococcus aureus Keratitis.
Host defense against bacterial keratitis.
Immunization with alpha-toxin toxoid protects the cornea against tissue damage during experimental Staphylococcus aureus keratitis.
Coronary Disease
Sphingomyelinase activity of LDL: a link between atherosclerosis, ceramide, and apoptosis?
COVID-19
Association between Functional Inhibitors of Acid Sphingomyelinase (FIASMAs) and Reduced Risk of Death in COVID-19 Patients: A Retrospective Cohort Study.
Pharmacological Inhibition of Acid Sphingomyelinase Prevents Uptake of SARS-CoV-2 by Epithelial Cells.
Repurposing antidepressants inhibiting the sphingomyelinase acid/ceramide system against COVID-19: current evidence and potential mechanisms.
SARS-CoV-2 infection induces the activation of tissue factor-mediated coagulation via activation of acid sphingomyelinase.
Update on Functional Inhibitors of Acid Sphingomyelinase (FIASMAs) in SARS-CoV-2 Infection.
Crohn Disease
The Clostridium perfringensalpha-toxin.
Cryptosporidiosis
Involvement of Cryptosporidium parvum Cdg7_FLc_1000 RNA in the Attenuation of Intestinal Epithelial Cell Migration via Trans-Suppression of Host Cell SMPD3.
Cystic Fibrosis
Accumulation of ceramide in the trachea and intestine of cystic fibrosis mice causes inflammation and cell death.
Acid Sphingomyelinase Inhibitors Normalize Pulmonary Ceramide and Inflammation in Cystic Fibrosis.
Antibodies to staphylococcal teichoic acid and alpha toxin in patients with cystic fibrosis.
Bacterial Sphingomyelinase is a State-Dependent Inhibitor of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR).
Ceramide in Pseudomonas aeruginosa infections and cystic fibrosis.
Ceramide mediates lung fibrosis in cystic fibrosis.
Defective Acid Sphingomyelinase Pathway with Pseudomonas aeruginosa Infection in Cystic Fibrosis.
Expression of intestinal and lung alkaline sphingomyelinase and neutral ceramidase in cystic fibrosis f508del transgenic mice.
Precision toxicology shows that troxerutin alleviates ochratoxin A-induced renal lipotoxicity.
The role of sphingolipids and ceramide in pulmonary inflammation in cystic fibrosis.
Therapeutic efficacy and safety of amitriptyline in patients with cystic fibrosis.
Cysts
TNF-alpha enhances Toxoplasma gondii cyst formation in human fibroblasts through the sphingomyelinase pathway.
Deafness
Partial sphingomyelinase deficiency with sea-blue histiocytosis and neurovisceral dysfunction.
Dehydration
Salinity Effects on Guard Cell Proteome in Chenopodium quinoa.
Dementia
Astrocytic ceramide as possible indicator of neuroinflammation.
SMPD1 variants do not have a major role in rapid eye movement sleep behavior disorder.
Sphingolipid metabolism correlates with cerebrospinal fluid Beta amyloid levels in Alzheimer's disease.
The Roles of Neutral Sphingomyelinases in Neurological Pathologies.
[Mutation del 1,02kb in the CLN3 gene and extrapyramidal syndrome].
Demyelinating Diseases
Acid sphingomyelinase deficiency enhances myelin repair after acute and chronic demyelination.
Dentinogenesis Imperfecta
A deletion in the gene encoding sphingomyelin phosphodiesterase 3 (Smpd3) results in osteogenesis and dentinogenesis imperfecta in the mouse.
Upregulation of smpd3 via BMP2 stimulation and Runx2.
Dermatitis
Clostridium perfringens alpha-toxin and NetB toxin antibodies and their possible role in protection against necrotic enteritis and gangrenous dermatitis in broiler chickens.
Dermatitis, Atopic
Alpha-toxin is produced by skin colonizing Staphylococcus aureus and induces a T helper type 1 response in atopic dermatitis.
Association analysis of sphingomyelinase 2 polymorphisms for the extrinsic type of atopic dermatitis in Koreans.
Effects of sphingomyelin/ceramide ratio on the permeability and microstructure of model stratum corneum lipid membranes.
Impaired sphingomyelinase activity and epidermal differentiation in atopic dermatitis.
Localization of sphingomyelinase in lesional skin of atopic dermatitis patients.
Skin of atopic dermatitis patients shows disturbed ?-glucocerebrosidase and acid sphingomyelinase activity that relates to changes in stratum corneum lipid composition.
Staphylococcal alpha-toxin is a strong inducer of interleukin-17 in humans.
Staphylococcal toxins and protein A differentially induce cytotoxicity and release of tumor necrosis factor-alpha from human keratinocytes.
Diabetes Complications
SMPDL3b modulates insulin receptor signaling in diabetic kidney disease.
Diabetes Mellitus
Genome-Wide DNA Methylation Profiles of Phlegm-Dampness Constitution.
Diabetes Mellitus, Experimental
Effect of streptozotocin-diabetes on the functioning of the sphingomyelin-signalling pathway in skeletal muscles of the rat.
Diabetes Mellitus, Type 2
ApoCIII-enriched LDL in type 2 diabetes displays altered lipid composition, increased susceptibility for sphingomyelinase, and increased binding to biglycan.
Secretory Zn2+-dependent sphingomyelinase activity in the serum of patients with type 2 diabetes is elevated.
Sphingomyelinases and Liver Diseases.
Diabetic Nephropathies
SMPDL3b modulates insulin receptor signaling in diabetic kidney disease.
Diabetic Neuropathies
Alpha-mangostin attenuates diabetic nephropathy in association with suppression of acid sphingomyelianse and endoplasmic reticulum stress.
Diabetic Retinopathy
Role of acid sphingomyelinase in shifting the balance between pro-inflammatory and reparative bone marrow cells in diabetic retinopathy.
Digestive System Diseases
Alkaline sphingomyelinase (NPP7) in hepatobiliary diseases: A field that needs to be closely studied.
Purification of a newly identified alkaline sphingomyelinase in human bile and effects of bile salts and phosphatidylcholine on enzyme activity.
Diphtheria
Comparative study of immune status to infectious agents in elderly patients with multiple myeloma, Waldenstrom's macroglobulinemia, and monoclonal gammopathy of undetermined significance.
The influence of bacterial exotoxins and endotoxins on the phagocytic activity of human macrophages in culture.
Uptake of fluorochrome-labelled exotoxins by different cell types in culture.
Use of silkworm larvae to study pathogenic bacterial toxins.
Drug-Related Side Effects and Adverse Reactions
Novel first-dose adverse drug reactions during a phase I trial of olipudase alfa (recombinant human acid sphingomyelinase) in adults with Niemann-Pick disease type B (acid sphingomyelinase deficiency).
Dwarfism
Neutral Sphingomyelinase (SMPD3) Deficiency Causes a Novel Form of Chondrodysplasia and Dwarfism That Is Rescued by Col2A1-Driven smpd3 Transgene Expression.
Neutral sphingomyelinase (SMPD3) deficiency causes a novel form of chondrodysplasia and dwarfism that is rescued by Col2A1-driven smpd3 transgene expression.
Neutral Sphingomyelinase 2 (SMPD3) Deficiency in Mice Causes Chondrodysplasia with Unimpaired Skeletal Mineralization.
Neutral sphingomyelinase 2 (smpd3) in the control of postnatal growth and development.
Encephalitis
DPTIP, a newly identified potent brain penetrant neutral sphingomyelinase 2 inhibitor, regulates astrocyte-peripheral immune communication following brain inflammation.
Encephalomyelitis
Acid sphingomyelinase deficiency increases susceptibility to fatal alphavirus encephalomyelitis.
Combination of Imipramine, a sphingomyelinase inhibitor, and ?-caryophyllene improve their therapeutic effects on experimental autoimmune encephalomyelitis (EAE).
Pharmacological Inhibition of Acid Sphingomyelinase Ameliorates Experimental Autoimmune Encephalomyelitis.
Encephalomyelitis, Autoimmune, Experimental
Combination of Imipramine, a sphingomyelinase inhibitor, and ?-caryophyllene improve their therapeutic effects on experimental autoimmune encephalomyelitis (EAE).
Pharmacological Inhibition of Acid Sphingomyelinase Ameliorates Experimental Autoimmune Encephalomyelitis.
Endocarditis
Hyperproduction of alpha-toxin by Staphylococcus aureus results in paradoxically reduced virulence in experimental endocarditis: a host defense role for platelet microbicidal proteins.
Regulation of Staphylococcus aureus alpha-toxin gene (hla) expression by agr, sarA, and sae in vitro and in experimental infective endocarditis.
Serological diagnosis of deep Staphylococcus aureus infections by enzyme-linked immunosorbent assay (ELISA) for staphylococcal hemolysins and teichoic acid.
Staphylococcal ?-Toxin Modulates Human Aortic Endothelial Cell and Platelet Function through Sphingomyelinase and Biofilm Ligase Activities.
Staphylococcus aureus ?-Toxin Mutants Are Defective in Biofilm Ligase and Sphingomyelinase Activity, and Causation of Infective Endocarditis/Sepsis.
Staphylococcus aureus ?-toxin Production is Common in Strains with the ?-toxin Gene Inactivated by Bacteriophage.
The clinical significance of serological methods in the diagnosis of staphylococcal septicaemia and endocarditis.
The role of antibodies against alpha-toxin and teichoic acid in the diagnosis of staphylococcal infections.
Virulence factors of Staphylococcus aureus in the pathogenesis of endocarditis. A comparative study of clinical isolates.
Endocarditis, Subacute Bacterial
Dual function of human IgA antibodies: inhibition of phagocytosis in circulating neutrophils and enhancement of responses in IL-8-stimulated cells.
Endometritis
Necrotizing endometritis and isolation of an alpha-toxin producing strain of Clostridium septicum in a wild sooty mangabey from Côte d'Ivoire.
Enteritis
A bivalent vaccine against avian necrotic enteritis and coccidiosis.
A Fast and Inexpensive Protocol for Empirical Verification of Neutralizing Epitopes in Microbial Toxins and Enzymes.
A new cytotoxin from Bacillus cereus that may cause necrotic enteritis.
A Recombinant Probiotic, Lactobacillus casei, Expressing the Clostridium perfringens ?-toxoid, as an Orally Vaccine Candidate Against Gas Gangrene and Necrotic Enteritis.
Alpha-toxin of Clostridium perfringens is not an essential virulence factor in necrotic enteritis in chickens.
Application of an Endothelial Cell Culture Assay for the Detection of Neutralizing Anti-Clostridium Perfringens Beta-Toxin Antibodies in a Porcine Vaccination Trial.
Binding studies on isolated porcine small intestinal mucosa and in vitro toxicity studies reveal lack of effect of C. perfringens beta-toxin on the porcine intestinal epithelium.
Clostridium perfringens alpha toxin affects electrophysiological properties of isolated jejunal mucosa of laying hens.
Clostridium perfringens alpha-toxin and NetB toxin antibodies and their possible role in protection against necrotic enteritis and gangrenous dermatitis in broiler chickens.
Clostridium perfringens beta-toxin targets endothelial cells in necrotizing enteritis in piglets.
Clostridium perfringens {beta}-toxin binding to vascular endothelial cells in a human case of enteritis necroticans.
Comparison of chicken gut colonisation by the pathogens Campylobacter jejuni and Clostridium perfringens by real-time quantitative PCR.
Diagnosis of Clostridium perfringens type C enteritis in pigs using a DNA amplification technique (PCR).
Epsilon-toxin production by Clostridium perfringens type D strain CN3718 is dependent upon the agr operon but not the VirS/VirR two-component regulatory system.
Highly conserved alpha-toxin sequences of avian isolates of Clostridium perfringens.
Identification and cloning of two immunogenic Clostridium perfringens proteins, elongation factor Tu (EF-Tu) and pyruvate:ferredoxin oxidoreductase (PFO) of C. perfringens.
Identification of changes in the composition of ileal bacterial microbiota of broiler chickens infected with Clostridium perfringens.
In vitro inhibitory effect of hen egg white lysozyme on Clostridium perfringens type A associated with broiler necrotic enteritis and its alpha-toxin production.
Involvement of tumour necrosis factor-alpha in Clostridium perfringens beta-toxin-induced plasma extravasation in mice.
Molecular docking analysis of P2X7 receptor with the beta toxin from Clostridium perfringens.
NetB, a New Toxin That Is Associated with Avian Necrotic Enteritis Caused by Clostridium perfringens.
Quantification of Cell Proliferation and Alpha-Toxin Gene Expression of Clostridium perfringens in the Development of Necrotic Enteritis in Broiler Chickens.
Rapid Cytopathic Effects of C. perfringens Beta-Toxin on Porcine Endothelial Cells.
Relative disease susceptibility and clostridial toxin antibody responses in three commercial broiler lines coinfected with Clostridium perfringens and Eimeria maxima using an experimental model of necrotic enteritis.
Role of P2X7 receptor in Clostridium perfringens beta-toxin-mediated cellular injury.
Sandwich ELISA detection of Clostridium perfringens cells and alpha-toxin from field cases of necrotic enteritis of poultry.
Sequence variation in the alpha-toxin encoding plc gene of Clostridium perfringens strains isolated from diseased and healthy chickens.
Susceptibility of primary human endothelial cells to C. perfringens beta-toxin suggesting similar pathogenesis in human and porcine necrotizing enteritis.
The Clostridium perfringensalpha-toxin.
The p38 MAPK and JNK Pathways Protect Host Cells against Clostridium perfringens Beta-Toxin.
Variable protection against experimental broiler necrotic enteritis after immunisation with the C-terminal fragment of Clostridium perfringens alpha-toxin and a non-toxic NetB variant.
[The necrotizing enteritis by Clostridium perfringens type C in piglets: II. Molecular epidemiology study]
Enterocolitis
Role of pannexin 1 in Clostridium perfringens beta-toxin-caused cell death.
Enterocolitis, Necrotizing
Clostridium septicum alpha-toxin forms pores and induces rapid cell necrosis.
Human meconium contains significant amounts of alkaline sphingomyelinase, neutral ceramidase, and sphingolipid metabolites.
Recent Insights into Clostridium perfringens Beta-Toxin.
Enterotoxemia
Clostridium perfringens toxin types in hooded seals in the Greenland Sea, determined by PCR and ELISA.
Epsilon-toxin production by Clostridium perfringens type D strain CN3718 is dependent upon the agr operon but not the VirS/VirR two-component regulatory system.
Recent Insights into Clostridium perfringens Beta-Toxin.
Role of P2X7 receptor in Clostridium perfringens beta-toxin-mediated cellular injury.
Role of pannexin 1 in Clostridium perfringens beta-toxin-caused cell death.
The enteric toxins of Clostridium perfringens.
The p38 MAPK and JNK Pathways Protect Host Cells against Clostridium perfringens Beta-Toxin.
Erythema
Acute bacterial skin and skin structure infection: Critical role of alpha-toxin and protective effects of its neutralization by a human antibody.
Brown spider phospholipase-D containing a conservative mutation (D233E) in the catalytic site: Identification and functional characterization.
Fabry Disease
A novel association between angiokeratoma corporis diffusum and acid sphingomyelinase deficiency.
Interstitial lung disease in lysosomal storage disorders.
Rapid screening for lipid storage disorders using biochemical markers. Expert center data and review of the literature.
Reconstruction of the Cytokine Signaling in Lysosomal Storage Diseases by Literature Mining and Network Analysis.
Farber Lipogranulomatosis
Acid Sphingomyelinase Deficiency Ameliorates Farber Disease.
My journey into the world of sphingolipids and sphingolipidoses.
On-tissue localization of ceramides and other sphingolipids by MALDI mass spectrometry imaging.
Fatty Liver
Acid sphingomyelinase deficiency in Western diet-fed mice protects against adipocyte hypertrophy and diet-induced liver steatosis.
Alcoholic and non-alcoholic fatty liver disease: Focus on ceramide.
ASMase regulates autophagy and lysosomal membrane permeabilization and its inhibition prevents early stage non-alcoholic steatohepatitis.
Metabolic therapy: lessons from liver diseases.
Sphingolipids and hepatic steatosis.
Fatty Liver, Alcoholic
Serum acid sphingomyelinase is upregulated in chronic hepatitis C infection and non alcoholic fatty liver disease.
Fibrosarcoma
Ceramide triggers p53-dependent apoptosis in genetically defined fibrosarcoma tumour cells.
Host acid sphingomyelinase regulates microvascular function not tumor immunity.
Role of Acid Sphingomyelinase and Ceramide in Mechano-Acoustic Enhancement of Tumor Radiation Responses.
Tumor response to radiotherapy regulated by endothelial cell apoptosis.
Foodborne Diseases
Clostridium Perfringens Bacteremia with Acute Hemolytic Anemia in the Setting of Endometrial Malignancy.
Isolation of Alpha-Toxin-Deficient Clostridium perfringens Type F from Sewage Influents and Effluents.
Preparation and characterization of a human scFv against the Clostridium perfringens type A alpha-toxin.
Frontotemporal Lobar Degeneration
Astrocytic ceramide as possible indicator of neuroinflammation.
Furunculosis
Sphingomyelinase activity of Staphylococcus aureus strains from recurrent furunculosis and other infections.
Gangliosidosis, GM1
Bi-functional IgG-lysosomal enzyme fusion proteins for brain drug delivery.
Ito cells in lysosomal storage disorders. An ultrastructural study.
Gas Gangrene
A cellular deficiency of gangliosides causes hypersensitivity to Clostridium perfringens phospholipase C.
A genetically engineered vaccine against the alpha-toxin of Clostridium perfringens protects mice against experimental gas gangrene.
A recombinant carboxy-terminal domain of alpha-toxin protects mice against Clostridium perfringens.
A Recombinant Probiotic, Lactobacillus casei, Expressing the Clostridium perfringens ?-toxoid, as an Orally Vaccine Candidate Against Gas Gangrene and Necrotic Enteritis.
Amentoflavone Attenuates Clostridium perfringens Gas Gangrene by Targeting Alpha-Toxin and Perfringolysin O.
Clostridial glucosylating toxins enter cells via clathrin-mediated endocytosis.
Clostridium absonum alpha-toxin: new insights into clostridial phospholipase C substrate binding and specificity.
Clostridium perfringens Alpha-Toxin Induces Gm1a Clustering and Trka Phosphorylation in the Host Cell Membrane.
Clostridium perfringens alpha-toxin induces rabbit neutrophil adhesion.
Clostridium perfringens Alpha-toxin Recognizes the GM1a/TrkA Complex.
Clostridium perfringens alpha-toxin: characterization and mode of action.
Clostridium Perfringens Bacteremia with Acute Hemolytic Anemia in the Setting of Endometrial Malignancy.
Clostridium septicum alpha-toxin forms pores and induces rapid cell necrosis.
Cross-complementation of Clostridium perfringens PLC and Clostridium septicum alpha-toxin mutants reveals PLC is sufficient to mediate gas gangrene.
Crystallization and preliminary X-ray diffraction studies of alpha-toxin from two different strains (NCTC8237 and CER89L43) of Clostridium perfringens.
Effect of erythromycin on biological activities induced by clostridium perfringens alpha-toxin.
Effects of Clostridium perfringens alpha-toxin (PLC) and perfringolysin O (PFO) on cytotoxicity to macrophages, on escape from the phagosomes of macrophages, and on persistence of C. perfringens in host tissues.
Effects of Clostridium perfringens phospholipase C in mammalian cells.
Identification of residues critical for toxicity in Clostridium perfringens phospholipase C, the key toxin in gas gangrene.
Isolation of alpha-toxin, theta-toxin and kappa-toxin mutants of Clostridium perfringens by Tn916 mutagenesis.
Large clostridial cytotoxins: cellular biology of Rho/Ras-glucosylating toxins.
Lipidomic profile of GM95 cell death induced by Clostridium perfringens alpha-toxin.
Membrane-Binding Mechanism of Clostridium perfringens Alpha-Toxin.
Perfringolysin O expression in Clostridium perfringens is independent of the upstream pfoR gene.
Phospholipase C and sphingomyelinase activities of the Clostridium perfringens alpha-toxin.
Preparation and characterization of a human scFv against the Clostridium perfringens type A alpha-toxin.
Role of tyrosine-57 and -65 in membrane-damaging and sphingomyelinase activities of Clostridium perfringens alpha-toxin.
Structure of the gangrene alpha-toxin: the beauty in the beast.
Structure of the key toxin in gas gangrene.
Synergistic effects of alpha-toxin and perfringolysin O in Clostridium perfringens-mediated gas gangrene.
The biochemistry of the gas gangrene toxins: 4. The reaction between the alpha-toxin (lecithinase) of Clostridium welchii and its antitoxin.
The biochemistry of the gas gangrene toxins; the reaction between the alpha-toxin, lecithinase, of Clostridium welchii and its antitoxin.
The Clostridium perfringensalpha-toxin.
The first strain of Clostridium perfringens isolated from an avian source has an alpha-toxin with divergent structural and kinetic properties.
The NanI and NanJ sialidases of Clostridium perfringens are not essential for virulence.
The Oncopathic Potency of Clostridium perfringens Is Independent of Its alpha-Toxin Gene.
The role of alpha-toxin of Clostridium perfringens in experimental gas gangrene in guinea pigs.
Use of genetically manipulated strains of Clostridium perfringens reveals that both alpha-toxin and theta-toxin are required for vascular leukostasis to occur in experimental gas gangrene.
Vaccines against Clostridium perfringens alpha-toxin.
Virulence studies on chromosomal alpha-toxin and theta-toxin mutants constructed by allelic exchange provide genetic evidence for the essential role of alpha-toxin in Clostridium perfringens-mediated gas gangrene.
[Characteristics of alpha-toxin of Clostridium perfringens and its role in the pathogenesis of gas gangrene (author's transl)]
[Toxigenesis of Clostridium onvyi type A. 1. Communication: production of alpha-toxin in vitro (author's transl)]
Gaucher Disease
Gaucher and Niemann-Pick diseases--enzymatic diagnosis in dried blood spots on filter paper: retrospective diagnoses in newborn-screening cards.
Interstitial lung disease in lysosomal storage disorders.
Plasma chitotriosidase and CCL18: early biochemical surrogate markers in type B Niemann-Pick disease.
Preliminary evidence for a processing error in the biosynthesis of Gaucher activator in mucolipidosis disease types II and III.
Prenatal diagnosis of Gaucher's and Niemann-Pick diseases. Assays of glucocerebrosidase and sphingomyelinase in tissue cultures using natural substrates.
Reconstruction of the Cytokine Signaling in Lysosomal Storage Diseases by Literature Mining and Network Analysis.
Studies on the activation of sphingomyelinase activity in Niemann-Pick type A, B, and C fibroblasts: enzymological differentiation of types A and B.
Genetic Diseases, Inborn
Combined Emphysema and Interstitial Lung Disease as a Rare Presentation of Pulmonary Involvement in a Patient with Chronic Visceral Acid Sphingomyelinase Deficiency (Niemann-Pick Disease Type B).
Connecting Hsp70, sphingolipid metabolism and lysosomal stability.
Disease manifestations and burden of illness in patients with acid sphingomyelinase deficiency (ASMD).
Identification and biochemical characterization of an acid sphingomyelinase-like protein from the bacterial plant pathogen Ralstonia solanacearum that hydrolyzes ATP to AMP but not sphingomyelin to ceramide.
Niemann-Pick disease type-B: a unique case report with compound heterozygosity and complicated lipid management.
Sperm Abnormalities in Heterozygous Acid Sphingomyelinase Knockout Mice Reveal a Novel Approach for the Prevention of Genetic Diseases.
Sperm abnormalities in heterozygous acid sphingomyelinase knockout mice reveal a novel approach for the prevention of genetic diseases.
Glioblastoma
Autophagy activation, lipotoxicity and lysosomal membrane permeabilization synergize to promote pimozide- and loperamide-induced glioma cell death.
Glioma
Acid sphingomyelinase activation requires caspase-8 but not p53 nor reactive oxygen species during Fas-induced apoptosis in human glioma cells.
Acidic sphingomyelinase: relationship with antidepressant-induced desensitization of beta-adrenoceptors.
Antidepressant-induced lipidosis with special reference to tricyclic compounds.
Calmodulin antagonist W-7 inhibits lysosomal sphingomyelinase activity in C6 glioma cells.
Ceramide metabolism determines glioma cell resistance to chemotherapy.
Channelling of intermediates in the biosynthesis of phosphatidylcholine and phosphatidylethanolamine in mammalian cells.
Effect of tricyclic antidepressants on sphingomyelinase and other sphingolipid hydrolases in C6 cultured glioma cells.
Influence of Bax or Bcl-2 overexpression on the ceramide-dependent apoptotic pathway in glioma cells.
Lysosomal ceramide mediates gemcitabine-induced death of glioma cells.
Modifications of sphingomyelin and phosphatidylcholine metabolism by tricyclic antidepressants and phenothiazines.
Neutral sphingomyelinase activation in endothelial and glial cell death induced by amyloid beta-peptide.
Overexpression of acid sphingomyelinase sensitizes glioma cells to chemotherapy.
p53 regulates ceramide formation by neutral sphingomyelinase through reactive oxygen species in human glioma cells.
p53-Independent ceramide formation in human glioma cells during gamma-radiation-induced apoptosis.
Penta-acetyl geniposide-induced apoptosis involving transcription of NGF/p75 via MAPK-mediated AP-1 activation in C6 glioma cells.
glucosylceramidase deficiency
Preliminary evidence for a processing error in the biosynthesis of Gaucher activator in mucolipidosis disease types II and III.
Graft vs Host Disease
Acid sphingomyelinase deficiency does not protect from graft-versus-host disease in transplant recipients with Niemann-Pick disease.
Bone marrow transplantation for Niemann-Pick type IA disease.
Successful hematopoietic stem cell transplantation for Niemann-Pick disease type B.
Granuloma
Fusogenicity of membranes: the impact of acid sphingomyelinase on innate immune responses.
Heart Failure
Adjustment of Dysregulated Ceramide Metabolism in a Murine Model of Sepsis-Induced Cardiac Dysfunction.
Biosynthesis of constitutive nitric oxide synthase-derived nitric oxide attenuates coronary vasoconstriction and myocardial depression in a model of septic heart failure induced by Staphylococcus aureus alpha-toxin.
Diaphragm dysfunction in heart failure is accompanied by increases in neutral sphingomyelinase activity and ceramide content.
Neutral sphingomyelinase: past, present and future.
Secretory sphingomyelinase is upregulated in chronic heart failure: a second messenger system of immune activation relates to body composition, muscular functional capacity, and peripheral blood flow.
Short-term magnesium deficiency downregulates telomerase, upregulates neutral sphingomyelinase and induces oxidative DNA damage in cardiovascular tissues: relevance to atherogenesis, cardiovascular diseases and aging.
Small hairpin RNA and pharmacological targeting of neutral sphingomyelinase prevents diaphragm weakness in rats with heart failure and reduced ejection fraction.
Sphingolipids in atherosclerosis and vascular biology.
Sphingomyelinase depresses force and calcium sensitivity of the contractile apparatus in mouse diaphragm muscle fibers.
Sphingomyelinase stimulates oxidant signaling to weaken skeletal muscle and promote fatigue.
Sphingomyelinases: their regulation and roles in cardiovascular pathophysiology.
The Polycomb group protein EED couples TNF receptor 1 to neutral sphingomyelinase.
Hepatitis
Acidic sphingomyelinase downregulates the liver-specific methionine adenosyltransferase 1A, contributing to tumor necrosis factor-induced lethal hepatitis.
Diagnosing Clostridium perfringens-associated necrotic enteritis in broiler flocks by an immunoglobulin G anti-alpha-toxin enzyme-linked immunosorbent assay.
Hepatitis C, Chronic
Serum acid sphingomyelinase is upregulated in chronic hepatitis C infection and non alcoholic fatty liver disease.
Hepatolenticular Degeneration
Liver cell death and anemia in Wilson disease involve acid sphingomyelinase and ceramide.
Herpes Simplex
Neutral sphingomyelinase-inhibiting guanidines prevent herpes simplex virus-1 replication.
Role of Sphingomyelin in Alphaherpesvirus Entry.
Herpes Zoster
Relationship between endotoxin core, staphylococcal and varicella antibody levels and outcome following aortic valve replacement surgery: a prospective observational study.
Histiocytosis
Pathogenesis of one variant of sea-blue histiocytosis.
Splenic histiocytosis in idiopathic thrombocytopenic purpura: a relative sphingomyelinase deficiency?
HIV Infections
Sphingomyelinase restricts the lateral diffusion of CD4 and inhibits human immunodeficiency virus fusion.
Hyperalgesia
Analgesic Effects of Lipid Raft Disruption by Sphingomyelinase and Myriocin via Transient Receptor Potential Vanilloid 1 and Transient Receptor Potential Ankyrin 1 Ion Channel Modulation.
Anti-allodynic effect of intracerebroventricularly administered antioxidant and free radical scavenger in a mouse model of orofacial pain.
The p75(NTR) Signaling Cascade Mediates Mechanical Hyperalgesia Induced By Nerve Growth Factor Injected Into The Rat Hind Paw.
Hypercholesterolemia
Endothelial NLRP3 inflammasome activation and arterial neointima formation associated with acid sphingomyelinase during hypercholesterolemia.
Hyperglycemia
Acid Sphingomyelinase Deficiency Prevents Diet-induced Hepatic Triacylglycerol Accumulation and Hyperglycemia in Mice.
Hyperhomocysteinemia
Acid sphingomyelinase gene knockout ameliorates hyperhomocysteinemic glomerular injury in mice lacking cystathionine-?-synthase.
Hyperinsulinism
Altered adipose and plasma sphingolipid metabolism in obesity: a potential mechanism for cardiovascular and metabolic risk.
Hyperlipidemias
Modulation of Lipid Metabolism by Celastrol.
Hyperlipoproteinemia Type II
Impairment of exogenous sphingomyelin degradation in cultured fibroblasts from familial hypercholesterolemia.
Hyperphosphatemia
Barley-ß-glucans reduce systemic inflammation, renal injury and aortic calcification through ADAM17 and neutral-sphingomyelinase2 inhibition.
Hypersensitivity
The common acid sphingomyelinase polymorphism p.G508R is associated with self-reported allergy.
Hypersensitivity, Delayed
[Characteristics of a staphylococcal infection in guinea pigs with delayed hypersensitivity to staphylococcal alpha-toxin]
Hypertension
Membrane rafts-redox signalling pathway contributes to renal fibrosis via modulation of the renal tubular epithelial-mesenchymal transition.
Short-term magnesium deficiency downregulates telomerase, upregulates neutral sphingomyelinase and induces oxidative DNA damage in cardiovascular tissues: relevance to atherogenesis, cardiovascular diseases and aging.
Staphylococcal alpha-toxin elicits hypertension in isolated rabbit lungs. Evidence for thromboxane formation and the role of extracellular calcium.
Staphylococcal alpha-toxin-induced vascular leakage in isolated perfused rabbit lungs.
Hypertension, Pulmonary
Endotoxin primes perfused rabbit lungs for enhanced vasoconstrictor response to staphylococcal alpha-toxin.
Hyperthyroidism
Long- and short-term effects of thyroxine on sphingolipid metabolism in rat liver.
Hypertriglyceridemia
Compound heterozygosity at the sphingomyelin phosphodiesterase-1 (SMPD1) gene is associated with low HDL cholesterol.
Impetigo
Staphylococcal scalded skin syndrome. II. Serum level of anti exfoliatin and anti alpha-toxin in patients with staphylococcal scalded skin syndrome or bullous impetigo.
Infarction, Middle Cerebral Artery
Homozygous Smpd1 deficiency aggravates brain ischemia/ reperfusion injury by mechanisms involving polymorphonuclear neutrophils, whereas heterozygous Smpd1 deficiency protects against mild focal cerebral ischemia.
Infections
A high-throughput sphingomyelinase assay using natural substrate.
Accumulation of aquaporin-1 during hemolysin-induced necrotic cell death.
Acid sphingomyelinase amplifies redox signaling in Pseudomonas aeruginosa-induced macrophage apoptosis.
Acid Sphingomyelinase Contributes to the Control of Mycobacterial Infection via a Signaling Cascade Leading from Reactive Oxygen Species to Cathepsin D.
Acid sphingomyelinase deficiency increases susceptibility to fatal alphavirus encephalomyelitis.
Acid Sphingomyelinase Inhibitors Normalize Pulmonary Ceramide and Inflammation in Cystic Fibrosis.
Acid Sphingomyelinase-Ceramide System in Bacterial Infections.
Acute bacterial skin and skin structure infection: Critical role of alpha-toxin and protective effects of its neutralization by a human antibody.
Alpha-toxin facilitates the generation of CXC chemokine gradients and stimulates neutrophil homing in Staphylococcus aureus pneumonia.
Alpha-toxin induces programmed cell death of human T cells, B cells, and monocytes during USA300 infection.
Alterations in ceramide concentration and pH determine the release of reactive oxygen species by Cftr-deficient macrophages on infection.
An acidic sphingomyelinase Type C activity from Mycobacterium tuberculosis.
Analysis of the effect of Sphingomyelinase on rubellavirus infectivity in two cell lines.
Anti-alpha-toxin monoclonal antibody and antibiotic combination therapy improves disease outcome and accelerates healing in a Staphylococcus aureus dermonecrosis model.
ASMase: the tailor of cytotoxic T cell granule exocytosis.
Association between antidepressant use and reduced risk of intubation or death in hospitalized patients with COVID-19: results from an observational study.
Bilateral Cystic Bronchiectasis as Novel Phenotype of Niemann-Pick Disease Type B Successfully Treated With Double Lung Transplantation.
Biological effects of the interaction of staphylococcal alpha-toxin with human serum.
Ceramide and sphingosine in pulmonary infections.
Ceramide in Pseudomonas aeruginosa infections and cystic fibrosis.
Ceramide plays a prominent role in MDA-7/IL-24-induced cancer-specific apoptosis.
Ceramide, a target for antiretroviral therapy.
Ceramide-enriched membrane domains.
Clostridium perfringens alpha toxin affects electrophysiological properties of isolated jejunal mucosa of laying hens.
Clostridium perfringens alpha-toxin: characterization and mode of action.
Co-option of Membrane Wounding Enables Virus Penetration into Cells.
Construction and virulence testing of a collagenase mutant of Clostridium perfringens.
Corneal virulence of Staphylococcus aureus: roles of alpha-toxin and protein A in pathogenesis.
Deciphering the Pathological Role of Staphylococcal ?-Toxin and Panton-Valentine Leukocidin Using a Novel Ex Vivo Human Skin Model.
Decreased plasma phospholipid concentrations and increased acid sphingomyelinase activity are accurate biomarkers for community-acquired pneumonia.
Decreasing SMPD1 activity in BEAS-2B bronchial airway epithelial cells results in increased NRF2 activity, cytokine synthesis and neutrophil recruitment.
Defective Acid Sphingomyelinase Pathway with Pseudomonas aeruginosa Infection in Cystic Fibrosis.
Depletion of Host and Viral Sphingomyelin Impairs Influenza Virus Infection.
Detection of neutralizing antibodies against alpha-toxin of different Clostridium septicum strains in cell culture.
Drosophila melanogaster as a model host for Staphylococcus aureus infection.
Ebolavirus requires acid sphingomyelinase activity and plasma membrane sphingomyelin for infection.
Effect of 2 Emulsion-Based Adjuvants on the Structure and Thermal Stability of Staphylococcus aureus Alpha-Toxin.
Effects of amoxicillin, gentamicin, and moxifloxacin on the hemolytic activity of Staphylococcus aureus in vitro and in vivo.
Effects of Clostridium perfringens alpha-toxin (PLC) and perfringolysin O (PFO) on cytotoxicity to macrophages, on escape from the phagosomes of macrophages, and on persistence of C. perfringens in host tissues.
Effects of Mycoplasma pneumoniae infection on sphingolipid metabolism in human lung carcinoma A549 cells.
Efficacy of a Multimechanistic Monoclonal Antibody Combination against Staphylococcus aureus Surgical Site Infections in Mice.
Enterotoxic Clostridia: Clostridium perfringens Enteric Diseases.
Evaluation of the Expression and Protective Potential of Leptospiral Sphingomyelinases.
Ex vivo assay to evaluate the efficacy of drugs targeting sphingolipids in preventing SARS-CoV-2 infection of nasal epithelial cells.
Exosomes mediate Zika virus transmission through SMPD3 neutral Sphingomyelinase in cortical neurons.
Expression of recombinant human acid sphingomyelinase in insect Sf21 cells: purification, processing and enzymatic characterization.
Factor associated with neutral sphingomyelinase activity mediates navigational capacity of leukocytes responding to wounds and infection: live imaging studies in zebrafish larvae.
Functional inhibition of acid sphingomyelinase disrupts infection by intracellular bacterial pathogens.
Functional inhibition or genetic deletion of acid sphingomyelinase bacteriostatically inhibits Anaplasma phagocytophilum infection in vivo.
Fusogenicity of membranes: the impact of acid sphingomyelinase on innate immune responses.
Group B streptococcus induces trophoblast death.
Hemolysin and K antigens in relation to serotype and hemagglutination type of Escherichia coli isolated from extraintestinal infections.
Heterogeneity of hemolysin expression during neonatal Streptococcus agalactiae sepsis.
High-level over-expression, purification, and crystallization of a novel phospholipase C/sphingomyelinase from Pseudomonas aeruginosa.
Host defense against Pseudomonas aeruginosa requires ceramide-rich membrane rafts.
Host sphingomyelin increases West Nile virus infection in vivo.
Immune recruitment and bactericidal activity of neutrophils in milk of cows vaccinated with staphylococcal alpha-toxin.
Immunohistochemical localization of Clostridium perfringens beta2-toxin in the gastrointestinal tract of horses.
Impact of the regulatory loci agr, sarA and sae of Staphylococcus aureus on the induction of alpha-toxin during device-related infection resolved by direct quantitative transcript analysis.
Incorporation of staphylococcal alpha-toxin in glutaraldehyde fixed erythrocytes.
Influence of amitriptyline on eryptosis, parasitemia and survival of Plasmodium berghei-infected mice.
Inhibition of acid sphingomyelinase by ambroxol prevents SARS-CoV-2 entry into epithelial cells.
Inhibition of chemokine receptor function by membrane cholesterol oxidation.
Inhibition of neutral sphingomyelinase protects mice against systemic tuberculosis.
Involvement of ceramide in the propagation of Japanese encephalitis virus.
Involvement of mitogen-activated protein kinases in Group B Streptococcus-induced macrophage apoptosis.
Isolation of alpha-toxin, theta-toxin and kappa-toxin mutants of Clostridium perfringens by Tn916 mutagenesis.
Keep Your Friends Close, but Your Enemies Closer: Role of Acid Sphingomyelinase During Infection and Host Response.
Leishmania-induced biphasic ceramide generation in macrophages is crucial for uptake and survival of the parasite.
Lipase versus teichoic acid and alpha-toxin as antigen in an enzyme immunoassay for serological diagnosis of Staphylococcus aureus infections.
Live attenuated vaccine-based control of necrotic enteritis of broiler chickens.
Local delivery of soluble interleukin-6 receptors to improve the outcome of alpha-toxin producing Staphylococcus aureus infection in mice.
Mechanism of leukotriene generation in polymorphonuclear leukocytes by staphylococcal alpha-toxin.
Membrane-Binding Mechanism of Clostridium perfringens Alpha-Toxin.
Modulation of enzymatic activity and biological function of Listeria monocytogenes broad-range phospholipase C by amino acid substitutions and by replacement with the Bacillus cereus ortholog.
Mycobacterial Infection is Promoted by Neutral Sphingomyelinase 2 Regulating a Signaling Cascade Leading to Activation of ?1-Integrin.
Novel inhibitor of bacterial sphingomyelinase, SMY-540, developed based on three-dimensional structure analysis.
Oligomer formation of Clostridium perfringens epsilon-toxin is induced by activation of neutral sphingomyelinase.
Pharmacological Inhibition of Acid Sphingomyelinase Prevents Uptake of SARS-CoV-2 by Epithelial Cells.
Precipitating antibodies against Staphylococcus aureus in experimental rabbit osteomyelitis, investigated by means of quantitative immunoelectrophoretic methods.
Production and applications of an N-terminally-truncated recombinant beta-haemolysin from Staphylococcus aureus.
Purification and characterization of neutral sphingomyelinase from Helicobacter pylori.
Raft ceramide in molecular medicine.
Recent Insights into Clostridium perfringens Beta-Toxin.
Regulated antisense RNA eliminates alpha-toxin virulence in Staphylococcus aureus infection.
Regulation of Staphylococcus aureus Infection of Macrophages by CD44, Reactive Oxygen Species, and Acid Sphingomyelinase.
Repurposing functional inhibitors of acid sphingomyelinase (fiasmas): an opportunity against SARS-CoV-2 infection?
Rhinoviruses infect human epithelial cells via ceramide-enriched membrane platforms.
Role of acid sphingomyelinase and IL-6 as mediators of endotoxin-induced pulmonary vascular dysfunction.
Role of alpha-toxin in Clostridium perfringens infection determined by using recombinants of C. perfringens and Bacillus subtilis.
Role of Sphingomyelin in Alphaherpesvirus Entry.
Role of Sphingomyelinase in the Pathogenesis of Bacillus cereus Infection.
SARS-CoV-2 infection induces the activation of tissue factor-mediated coagulation via activation of acid sphingomyelinase.
Serum acid sphingomyelinase is upregulated in chronic hepatitis C infection and non alcoholic fatty liver disease.
Specific roles of alpha-toxin and beta-toxin during Staphylococcus aureus corneal infection.
Sphingomyelinase activity of Staphylococcus aureus strains from recurrent furunculosis and other infections.
Staphylococcal alpha-toxin-induced vascular leakage in isolated perfused rabbit lungs.
Staphylococcus aureus ?-toxin Production is Common in Strains with the ?-toxin Gene Inactivated by Bacteriophage.
Staphylococcus aureus Alpha-Toxin Limits Type 1 While Fostering Type 3 Immune Responses.
Staphylococcus aureus alpha-toxin mediates general and cell type-specific changes in metabolite concentrations of immortalized human airway epithelial cells.
Staphylococcus aureus Biofilms Induce Macrophage Dysfunction Through Leukocidin AB and Alpha-Toxin.
Staphylococcus aureus in chronic and recurrent infections.
Staphylococcus corneal virulence in a new topical model of infection.
Streptococcus iniae beta-hemolysin streptolysin S is a virulence factor in fish infection.
Sub-Inhibitory Concentrations of Mupirocin Strongly Inhibit Alpha-Toxin Production in High-Level Mupirocin-Resistant MRSA by Down-Regulating agr, saeRS, and sarA.
Synergistic effects of alpha-toxin and perfringolysin O in Clostridium perfringens-mediated gas gangrene.
T Cell-Specific Overexpression of Acid Sphingomyelinase Results in Elevated T Cell Activation and Reduced Parasitemia During Plasmodium yoelii Infection.
The association of CD81 with tetraspanin-enriched microdomains is not essential for Hepatitis C virus entry.
The H35A mutated alpha-toxin interferes with cytotoxicity of staphylococcal alpha-toxin.
The psm? locus regulates production of Staphylococcus aureus alpha-toxin during infection.
The role of antibodies against alpha-toxin and teichoic acid in the diagnosis of staphylococcal infections.
The Role of hlb-Converting Bacteriophages in Staphylococcus aureus Host Adaption.
The role of humoral immunity and acute inflammation in protection against staphyloccocal dermonecrosis.
Therapeutic efficacy and safety of amitriptyline in patients with cystic fibrosis.
Therapy of CF-patients with amitriptyline and placebo--a randomised, double-blind, placebo-controlled phase IIb multicenter, cohort-study.
Trypanosoma cruzi modulates gene expression of plasma membrane repair-related proteins.
Vaccines against Clostridium perfringens alpha-toxin.
Zika virus propagation and release in human fetal astrocytes can be suppressed by neutral sphingomyelinase-2 inhibitor GW4869.
[Structure and function of sphingomyelinase]
[The necrotizing enteritis by Clostridium perfringens type C in piglets: practical observations, control and epidemiology]
Inflammatory Bowel Diseases
Acid sphingomyelinase inhibition suppresses lipopolysaccharide-mediated release of inflammatory cytokines from macrophages and protects against disease pathology in dextran sulphate sodium-induced colitis in mice.
Alkaline sphingomyelinase: an old enzyme with novel implications.
Boswellic acid inhibits expression of acid sphingomyelinase in intestinal cells.
Inhibition of lipopolysaccharide-induced release of interleukin-8 from intestinal epithelial cells by SMA, a novel inhibitor of sphingomyelinase and its therapeutic effect on dextran sulphate sodium-induced colitis in mice.
Ursolic Acid Inhibits Acid Sphingomyelinase in Intestinal Cells.
Influenza, Human
Differential role of sphingomyelin in influenza virus, rhinovirus and SARS-CoV-2 infection of Calu-3 cells.
Linezolid Attenuates Lethal Lung Damage during Postinfluenza Methicillin-Resistant Staphylococcus aureus Pneumonia.
Targeting the endolysosomal host-SARS-CoV-2 interface by clinically licensed functional inhibitors of acid sphingomyelinase (FIASMA) including the antidepressant fluoxetine.
Insulin Resistance
Acid Sphingomyelinase Deficiency Prevents Diet-induced Hepatic Triacylglycerol Accumulation and Hyperglycemia in Mice.
Acid Sphingomyelinase Down-regulation Alleviates Vascular Endothelial Insulin Resistance in Diabetic Rats.
Ceramide content is increased in skeletal muscle from obese insulin-resistant humans.
Common inhibitory serine sites phosphorylated by IRS-1 kinases, triggered by insulin and inducers of insulin resistance.
Genome-Wide DNA Methylation Profiles of Phlegm-Dampness Constitution.
High fat diet induced hepatic steatosis and insulin resistance: Role of dysregulated ceramide metabolism.
Inhibition of neutral sphingomyelinases in skeletal muscle attenuates fatty-acid induced defects in metabolism and stress.
Sortilin deficiency improves the metabolic phenotype and reduces hepatic steatosis of mice subjected to diet-induced obesity.
Tumor necrosis factor-alpha, sphingomyelinase and ceramides activate tyrosine kinase, p21Ras and phosphatidylinositol 3-kinase: implications for glucose transport and insulin resistance.
[ROLE OF NEUTRAL SPHINGOMYELINASE IN AGE-DEPENDENT MUSCLE INSULIN RESISTANCE DEVELOPMENT AND ITS IMPROVEMENT WITH N-ACETYLCYSTEINE].
Insulinoma
The acid sphingomyelinase inhibitor SR33557 counteracts TNF-alpha-mediated potentiation of IL-1beta-induced NF-kappaB activation in the insulin-producing cell line Rinm5F.
The group VIA calcium-independent phospholipase A2 participates in ER stress-induced INS-1 insulinoma cell apoptosis by promoting ceramide generation via hydrolysis of sphingomyelins by neutral sphingomyelinase.
Intellectual Disability
Chronic Niemann-Pick disease with sphingomyelinase deficiency in two brothers with mental retardation.
Synaptic vesicle docking: sphingosine regulates syntaxin1 interaction with Munc18.
Intestinal Diseases
Immunohistochemical localization of Clostridium perfringens beta2-toxin in the gastrointestinal tract of horses.
Intestinal Neoplasms
Detection of alkaline sphingomyelinase activity in human stool: proposed role as a new diagnostic and prognostic marker of colorectal cancer.
Regulatory T cells reduce endothelial neutral sphingomyelinase 2 to prevent T-cell migration into tumors.
Intraabdominal Infections
Candida albicans Impacts Staphylococcus aureus Alpha-Toxin Production via Extracellular Alkalinization.
Iritis
Specific roles of alpha-toxin and beta-toxin during Staphylococcus aureus corneal infection.
Keratitis
Age-Related Differences in Rabbits during Experimental Staphylococcus aureus Keratitis.
Comparison of potential pathogenic traits of staphylococci that may contribute to corneal ulceration and inflammation.
Corneal pathogenesis of Staphylococcus aureus strain Newman.
Corneal virulence of Staphylococcus aureus: roles of alpha-toxin and protein A in pathogenesis.
Effectiveness of Alpha-toxin Fab Monoclonal Antibody Therapy in Limiting the Pathology of Staphylococcus aureus Keratitis.
Effects of toxin production in a murine model of Staphylococcus aureus keratitis.
Histopathological studies of staphylococcal alpha-toxin: effects on rabbit corneas.
Host defense against bacterial keratitis.
Immunization with alpha-toxin toxoid protects the cornea against tissue damage during experimental Staphylococcus aureus keratitis.
Specific roles of alpha-toxin and beta-toxin during Staphylococcus aureus corneal infection.
Staphylococcus corneal virulence in a new topical model of infection.
Kidney Diseases
Identification and biochemical characterization of an acid sphingomyelinase-like protein from the bacterial plant pathogen Ralstonia solanacearum that hydrolyzes ATP to AMP but not sphingomyelin to ceramide.
Leptospirosis
Cytotoxicity of the 42 kDa SMase C sphingomyelinase secreted by Leptospira interrogans serovar Pomona on Vero cells.
Evaluation of the Expression and Protective Potential of Leptospiral Sphingomyelinases.
Leukemia
Aggregation of IgE receptors induces degranulation in rat basophilic leukemia cells permeabilized with alpha-toxin from Staphylococcus aureus.
Analysis of receptor-G protein interactions in permeabilized cells.
Apoptotic effects of selected strains of lactic acid bacteria on a human T leukemia cell line are associated with bacterial arginine deiminase and/or sphingomyelinase activities.
ATP-dependent activation of phospholipase C by antigen, NECA, Na3VO4, and GTP-gamma-S in permeabilized RBL cell ghosts: differential augmentation by ATP, phosphoenolpyruvate and phosphocreatine.
Ceramide 1-phosphate, a novel phospholipid in human leukemia (HL-60) cells. Synthesis via ceramide from sphingomyelin.
Ceramide generation during curcumin-induced apoptosis is controlled by crosstalk among Bcl-2, Bcl-xL, caspases and glutathione.
Fc epsilon RI-stimulated Ca(2+)-dependent secretion from rat basophilic leukemia (RBL-2H3) cells permeabilized with Staphylococcal alpha-toxin: Fc epsilon RI-operated signals are not mimicked by the actions of GTP gamma S.
Glutathione regulation of neutral sphingomyelinase in tumor necrosis factor-alpha-induced cell death.
Hexadecylphosphocholine does not influence phospholipase D and sphingomyelinase activity in human leukemia cells.
Holotoxin A? Induces Apoptosis by Activating Acid Sphingomyelinase and Neutral Sphingomyelinase in K562 and Human Primary Leukemia Cells.
Implications of sphingosine kinase 1 expression level for the cellular sphingolipid rheostat: relevance as a marker for daunorubicin sensitivity of leukemia cells.
Malignant fibrous histiocytoma-pleomorphic sarcoma, NOS gene expression, histology, and clinical course. A pilot study.
Mutations in the neutral sphingomyelinase gene SMPD3 implicate the ceramide pathway in human leukemias.
Resveratrol-induced transcriptional up-regulation of ASMase (SMPD1) of human leukemia and cancer cells.
Role of c-jun expression increased by heat shock- and ceramide-activated caspase-3 in HL-60 cell apoptosis. Possible involvement of ceramide in heat shock-induced apoptosis.
Role of sphingomyelin-MAPKs pathway in heat-induced apoptosis.
Stimulation of CD95-induced apoptosis in T-cells by a subtype specific neutral sphingomyelinase inhibitor.
Transbilayer movement of ceramide in the plasma membrane of live cells.
Up-regulation of acid sphingomyelinase during retinoic acid-induced myeloid differentiation of NB4, a human acute promyelocytic leukemia cell line.
Withanolide D induces apoptosis in leukemia by targeting the activation of neutral sphingomyelinase-ceramide cascade mediated by synergistic activation of c-Jun N-terminal kinase and p38 mitogen-activated protein kinase.
Leukemia, Lymphoid
Mutations in the neutral sphingomyelinase gene SMPD3 implicate the ceramide pathway in human leukemias.
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Green tea polyphenol epigallocatechin-O-gallate induces cell death by acid sphingomyelinase activation in chronic myeloid leukemia cells.
Leukemia, Myeloid
Positive feedback control of neutral sphingomyelinase activity by ceramide.
Role of ceramide as a lipid mediator of 1 alpha,25-dihydroxyvitamin D3-induced HL-60 cell differentiation.
The phosphoinositide 3-kinase/Akt pathway is activated by daunorubicin in human acute myeloid leukemia cell lines.
Leukemia, Myeloid, Acute
Mutations in the neutral sphingomyelinase gene SMPD3 implicate the ceramide pathway in human leukemias.
SMPDL3B Predicts Poor Prognosis and Contributes to Development of Acute Myeloid Leukemia.
Leukemia, Promyelocytic, Acute
Up-regulation of acid sphingomyelinase during retinoic acid-induced myeloid differentiation of NB4, a human acute promyelocytic leukemia cell line.
Leukocytosis
Inhibition of normal granulopoiesis by cytostatic agents.
Leukostasis
Cross-complementation of Clostridium perfringens PLC and Clostridium septicum alpha-toxin mutants reveals PLC is sufficient to mediate gas gangrene.
Use of genetically manipulated strains of Clostridium perfringens reveals that both alpha-toxin and theta-toxin are required for vascular leukostasis to occur in experimental gas gangrene.
Lipidoses
Adult sphingomyelinase deficiency: report of 2 patients who initially presented with psychiatric disorders.
Calmodulin antagonists chlorpromazine and W-7 inhibit exogenous cholesterol esterification and sphingomyelinase activity in human skin fibroblast cultures. Similarities between drug-induced and Niemann-Pick type C lipidoses.
Cocaine induces a mixed lysosomal lipidosis in cultured fibroblasts, by inactivation of acid sphingomyelinase and inhibition of phospholipase A1.
Deficiency of phospholipase C acting on phosphatidylglycerol in Niemann-Pick disease.
Deficiency of taurocholate-dependent phospholipase C acting on phosphatidylcholine in Niemann-Pick disease.
Enhancement of ricin A chain immunotoxin activity by perhexiline on established and fresh leukemic cells.
In vitro and in vivo enhancement of ricin-A chain immunotoxin activity by novel indolizine calcium channel blockers: delayed intracellular degradation linked to lipidosis induction.
Niemann-Pick disease and juvenile xanthogranuloma. Are they related?
Partial sphingomyelinase deficiency with sea-blue histiocytosis and neurovisceral dysfunction.
Reduction of acid sphingomyelinase activity in human fibroblasts induced by AY-9944 and other cationic amphiphilic drugs.
[Lipidosis with vertical gaze palsy, macular degeneration, and sphingomyelinase deficiency]
[Niemann-Pick disease type C (subacute neurovisceral lipidosis). Problems of altered sphingomyelinase activity in the brain (author's transl)]
Liver Cirrhosis
Acidic sphingomyelinase controls hepatic stellate cell activation and in vivo liver fibrogenesis.
Cathepsin B overexpression due to acid sphingomyelinase ablation promotes liver fibrosis in Niemann-Pick disease.
[Expression of ASMase in alcoholic liver fibrosis in rats].
Liver Diseases
Acid Sphingomyelinase Inhibition Prevents Development of Sepsis Sequelae in the Murine Liver.
Acidic sphingomyelinase downregulates the liver-specific methionine adenosyltransferase 1A, contributing to tumor necrosis factor-induced lethal hepatitis.
Alcoholic and non-alcoholic fatty liver disease: Focus on ceramide.
Cathepsin B overexpression due to acid sphingomyelinase ablation promotes liver fibrosis in Niemann-Pick disease.
Metabolic therapy: lessons from liver diseases.
Serum acid sphingomyelinase is upregulated in chronic hepatitis C infection and non alcoholic fatty liver disease.
Sphingomyelinases and Liver Diseases.
Liver Diseases, Alcoholic
Alcoholic liver disease: ASMase implicated in alcoholic liver disease.
Mitochondrial cholesterol accumulation in alcoholic liver disease: Role of ASMase and endoplasmic reticulum stress.
Liver Failure
Acidic sphingomyelinase downregulates the liver-specific methionine adenosyltransferase 1A, contributing to tumor necrosis factor-induced lethal hepatitis.
Group B streptococcal beta-hemolysin induces mortality and liver injury in experimental sepsis.
Liver Neoplasms
Acid sphingomyelinase is induced by butyrate but does not initiate the anticancer effect of butyrate in HT29 and HepG2 cells.
Recombinant human acid sphingomyelinase as an adjuvant to sorafenib treatment of experimental liver cancer.
Lung Diseases
Abnormal methylation of seven genes and their associations with clinical characteristics in early stage non-small cell lung cancer.
Development of carbohydrate-derived inhibitors of acid sphingomyelinase.
Inositol-trisphosphate reduces alveolar apoptosis and pulmonary edema in neonatal lung injury.
Quantitative Systems Pharmacology Modeling of Acid Sphingomyelinase Deficiency and the Enzyme Replacement Therapy Olipudase Alfa Is an Innovative Tool for Linking Pathophysiology and Pharmacology.
[Dynamics of the indices of natural resistance and of the response to staphylococcal alpha-toxin in acute suppurative lung diseases]
Lung Diseases, Interstitial
Bilateral Cystic Bronchiectasis as Novel Phenotype of Niemann-Pick Disease Type B Successfully Treated With Double Lung Transplantation.
Combined Emphysema and Interstitial Lung Disease as a Rare Presentation of Pulmonary Involvement in a Patient with Chronic Visceral Acid Sphingomyelinase Deficiency (Niemann-Pick Disease Type B).
Interstitial lung disease in lysosomal storage disorders.
Lung Injury
Acid Sphingomyelinase Inhibition Attenuates Cell Death in Mechanically-Ventilated Newborn Rat Lung.
Acid sphingomyelinase mediates murine acute lung injury following transfusion of aged platelets.
Extracellular Sphingomyelinase Rv0888 of Mycobacterium tuberculosis Contributes to Pathological Lung Injury of Mycobacterium smegmatis in Mice via Inducing Formation of Neutrophil Extracellular Traps.
Inhibition of Neutrophil Apoptosis via Sphingolipid Signaling in Acute Lung Injury.
Neutral Sphingomyelinase 2: A Novel Target in Cigarette Smoke-induced Apoptosis and Lung Injury.
nSMase2 activation and trafficking are modulated by oxidative stress to induce apoptosis.
Reevaluation of Lung Injury in TNF-Induced Shock: The Role of the Acid Sphingomyelinase.
Role of eicosanoids in staphylococcal alpha-toxin-induced lung injury in the rat.
Src Regulates Cigarette Smoke-induced Ceramide Generation via nSMase2 in the Airway Epithelium.
Staphylococcus aureus beta-toxin induces lung injury through syndecan-1.
The role of group B streptococci beta-hemolysin expression in newborn lung injury.
Lung Neoplasms
Abnormal methylation of seven genes and their associations with clinical characteristics in early stage non-small cell lung cancer.
Bilateral synchronous multiple lung nodules: Surgical experience from two cases.
Ceramide mediates nanovesicle shedding and cell death in response to phosphatidylinositol ether lipid analogs and perifosine.
Characteristics and prognosis of synchronous multiple primary lung cancer after surgical treatment: A systematic review and meta-analysis of current evidence.
Characterizing the sphingomyelinase pathway triggered by PRIMA-1 derivatives in lung cancer cells with differing p53 status.
Early clinical diagnosis of synchronous multiple primary lung cancer.
Lung cancer and lung injury: the dual role of ceramide.
Neutral sphingomyelinase-2, acid sphingomyelinase, and ceramide levels in COPD patients compared to controls.
Novel quinuclidinone derivatives induce apoptosis in lung cancer via sphingomyelinase pathways.
Preoperative virtual simulation for synchronous multiple primary lung cancers using three-dimensional computed tomography lung reconstruction: a case report.
Single-staged uniportal VATS major pulmonary resection for bilateral synchronous multiple primary lung cancers.
Surgical Prognosis of Synchronous Multiple Primary Lung Cancer: Systematic Review and Meta-Analysis.
Surgical Results of Synchronous Multiple Primary Lung Cancers: Similar to the Stage-Matched Solitary Primary Lung Cancers?
Surgical treatment of synchronous multiple primary lung cancers: a retrospective analysis of 122 patients.
Synchronous Bilateral Lung Cancer With Discordant Histology.
Unexpected encounters: high prevalence of synchronous primary lung cancers in a tertiary academic center.
Uniportal video-assisted thoracoscopic S8 segmentectomy and S1a subsegmentectomy for synchronous multiple primary lung cancers.
[Analysis on the Prognostic and Survival Factors of Synchronous Multiple Primary Lung Cancer].
Lymphadenopathy
Subclinical course of adult visceral Niemann-Pick type C1 disease. A rare or underdiagnosed disorder?
Lymphatic Metastasis
Surgical Prognosis of Synchronous Multiple Primary Lung Cancer: Systematic Review and Meta-Analysis.
Lymphocytic Choriomeningitis
Acid sphingomyelinase is a key regulator of cytotoxic granule secretion by primary T lymphocytes.
Fusogenicity of membranes: the impact of acid sphingomyelinase on innate immune responses.
Lymphohistiocytosis, Hemophagocytic
Elevated sphingomyelinase and hypercytokinemia in hemophagocytic lymphohistiocytosis.
Evaluation of the role of secretory sphingomyelinase and bioactive sphingolipids as biomarkers in hemophagocytic lymphohistiocytosis.
Lymphoma
Lysosomal ceramide generated by acid sphingomyelinase triggers cytosolic cathepsin B-mediated degradation of X-linked inhibitor of apoptosis protein in natural killer/T lymphoma cell apoptosis.
Regulatory roles of cell surface sialylation in susceptibility to sphingomyelinase in human diffuse large B cell lymphoma.
Lymphoma, B-Cell
Regulatory roles of cell surface sialylation in susceptibility to sphingomyelinase in human diffuse large B cell lymphoma.
Lymphoma, Large B-Cell, Diffuse
Regulatory roles of cell surface sialylation in susceptibility to sphingomyelinase in human diffuse large B cell lymphoma.
Lymphoma, T-Cell
Stimulation of CD95-induced apoptosis in T-cells by a subtype specific neutral sphingomyelinase inhibitor.
Lymphoma, T-Cell, Cutaneous
Staphylococcal alpha-toxin tilts the balance between malignant and non-malignant CD4+ T cells in cutaneous T-cell lymphoma.
Staphylococcus aureus alpha-toxin inhibits CD8+ T cell-mediated killing of cancer cells in cutaneous T-cell lymphoma.
Lysosomal Storage Diseases
An induced pluripotent stem cell line (TRNDi009-C) from a Niemann-Pick disease type A patient carrying a heterozygous p.L302P (c.905 T>C) mutation in the SMPD1 gene.
Autopsy pathology of infantile neurovisceral ASMD (Niemann-Pick Disease type A): Clinicopathologic correlations of a case report.
Burden of Illness in Acid Sphingomyelinase Deficiency: A Retrospective Chart Review of 100 Patients.
Cathepsin B overexpression due to acid sphingomyelinase ablation promotes liver fibrosis in Niemann-Pick disease.
Cholesterol trapping in Niemann-Pick disease type B fibroblasts can be relieved by expressing the phosphotyrosine binding domain of GULP.
Clathrin-mediated endocytosis is impaired in type A-B Niemann-Pick disease model cells and can be restored by ICAM-1-mediated enzyme replacement.
Consensus recommendation for a diagnostic guideline for acid sphingomyelinase deficiency.
Control of endothelial targeting and intracellular delivery of therapeutic enzymes by modulating the size and shape of ICAM-1-targeted carriers.
Defective Autophagy, Mitochondrial Clearance and Lipophagy in Niemann-Pick Type B Lymphocytes.
Disease manifestations and burden of illness in patients with acid sphingomyelinase deficiency (ASMD).
Functional characterization of novel variants in SMPD1 in Indian patients with acid sphingomyelinase deficiency.
Generation of an induced pluripotent stem cell line (TRNDi004-I) from a Niemann-Pick disease type B patient carrying a heterozygous mutation of p.L43_A44delLA in the SMPD1 gene.
Induced Pluripotent Stem Cells for Disease Modeling and Evaluation of Therapeutics for Niemann-Pick Disease Type A.
Lack of Acid Sphingomyelinase Induces Age-Related Retinal Degeneration.
Merits of Combination Cortical, Subcortical, and Cerebellar Injections for the Treatment of Niemann-Pick Disease Type A.
Mitochondrial biogenesis is transcriptionally repressed in lysosomal lipid storage diseases.
Ophthalmologic manifestations of type B Niemann-Pick diseases.
Polyneuropathy in feline Niemann-Pick disease.
Rapid screening for lipid storage disorders using biochemical markers. Expert center data and review of the literature.
Recommendations for clinical monitoring of patients with acid sphingomyelinase deficiency (ASMD).
Sphingomyelin lipidosis (Niemann-Pick disease) in a juvenile raccoon (Procyon lotor).
The contribution of Niemann-Pick SMPD1 mutations to Parkinson disease in Ashkenazi Jews.
[Mutation del 1,02kb in the CLN3 gene and extrapyramidal syndrome].
Macular Degeneration
Cholesterol-mediated activation of acid sphingomyelinase disrupts autophagy in the retinal pigment epithelium.
Protective responses to sublytic complement in the retinal pigment epithelium.
[Lipidosis with vertical gaze palsy, macular degeneration, and sphingomyelinase deficiency]
Magnesium Deficiency
Short-term magnesium deficiency downregulates telomerase, upregulates neutral sphingomyelinase and induces oxidative DNA damage in cardiovascular tissues: relevance to atherogenesis, cardiovascular diseases and aging.
Malaria
Influence of amitriptyline on eryptosis, parasitemia and survival of Plasmodium berghei-infected mice.
Neutral sphingomyelinase activity dependent on Mg2+ and anionic phospholipids in the intraerythrocytic malaria parasite Plasmodium falciparum.
Phospholipid organization in monkey erythrocytes upon Plasmodium knowlesi infection.
Mastitis
Conversion of chronic staphylococcal mastitis to acute gangrenous mastitis after neutropenia in blood and bone marrow produced by an equine anti-bovine leukocyte serum.
Effect immunization with highly purified alpha- and beta-toxins on staphylococcal mastitis in rabbits.
Geographical variation in the presence of genes encoding superantigenic exotoxins and beta-hemolysin among Staphylococcus aureus isolated from bovine mastitis in Europe and USA.
Identification of nonlipophilic corynebacteria isolated from dairy cows with mastitis.
Immunogenicity of alpha-toxin, capsular polysaccharide (CPS) and recombinant fibronectin-binding protein (r-FnBP) of Staphylococcus aureus in rabbit.
Measles
Ebolavirus requires acid sphingomyelinase activity and plasma membrane sphingomyelin for infection.
Neutral sphingomyelinase in physiological and measles virus induced T cell suppression.
Medulloblastoma
Off-target function of the Sonic hedgehog inhibitor cyclopamine in mediating apoptosis via nitric oxide-dependent neutral sphingomyelinase 2/ceramide induction.
Melanoma
A novel visible range FRET probe for monitoring acid sphingomyelinase activity in living cells.
Acid sphingomyelinase determines melanoma progression and metastatic behaviour via the microphtalmia-associated transcription factor signalling pathway.
Acid Sphingomyelinase Downregulation Enhances Mitochondrial Fusion and Promotes Oxidative Metabolism in a Mouse Model of Melanoma.
Acidic extracellular pH increases calcium influx-triggered phospholipase D activity along with acidic sphingomyelinase activation to induce matrix metalloproteinase-9 expression in mouse metastatic melanoma.
BETA-GALACTOSYLCERAMIDASE PROMOTES MELANOMA GROWTH VIA MODULATION OF CERAMIDE METABOLISM.
Essential role for acid sphingomyelinase-inhibited autophagy in melanoma response to cisplatin.
Melanogenesis in murine B16 cells exposed to Aeromonas hydrophila cytotoxic enterotoxin.
Melanoma cell metastasis via P-selectin-mediated activation of acid sphingomyelinase in platelets.
Modulation of Acid Sphingomyelinase in Melanoma Reprogrammes the Tumour Immune Microenvironment.
Neutral Sphingomyelinase 2 Heightens Anti-Melanoma Immune Responses and Anti-PD-1 Therapy Efficacy.
Nitric oxide boosts chemoimmunotherapy via inhibition of acid sphingomyelinase in a mouse model of melanoma.
Purification, molecular cloning, and application of a novel sphingomyelin-binding protein (clamlysin) from the brackishwater clam, Corbicula japonica.
Regulation of hematogenous tumor metastasis by acid sphingomyelinase.
Regulation of UDP-glucose:ceramide glucosyltransferase-1 by ceramide.
Role of Acid Sphingomyelinase-Induced Signaling in Melanoma Cells for Hematogenous Tumor Metastasis.
Tumor response to radiotherapy regulated by endothelial cell apoptosis.
Melanoma, Experimental
Acidic extracellular pH increases calcium influx-triggered phospholipase D activity along with acidic sphingomyelinase activation to induce matrix metalloproteinase-9 expression in mouse metastatic melanoma.
Host acid sphingomyelinase regulates microvascular function not tumor immunity.
Regulation of intracellular ceramide content in B16 melanoma cells. Biological implications of ceramide glycosylation.
Memory Disorders
Pharmacological reversion of sphingomyelin-induced dendritic spine anomalies in a Niemann Pick disease type A mouse model.
The Fountain of Youth: It's All in Our Veins.
Meningioma
Phospholipids and acidic sphingomyelinase in human meningiomas.
Mesothelioma
alpha-Toxin of Staphylococcus aureus overcomes acquired cisplatin-resistance in malignant mesothelioma cells.
Mesothelioma, Malignant
alpha-Toxin of Staphylococcus aureus overcomes acquired cisplatin-resistance in malignant mesothelioma cells.
Metabolic Diseases
Acid Sphingomyelinase Down-regulation Alleviates Vascular Endothelial Insulin Resistance in Diabetic Rats.
[Study of thesaurismosis induced by perhexiline maleate. Confirmation of experimental data]
Microcephaly
Exosomes mediate Zika virus transmission through SMPD3 neutral Sphingomyelinase in cortical neurons.
Loss of SMPD4 Causes a Developmental Disorder Characterized by Microcephaly and Congenital Arthrogryposis.
Mucocutaneous Lymph Node Syndrome
Elevation of Serum Acid Sphingomyelinase Activity in Acute Kawasaki Disease.
Mucolipidoses
Biosynthesis of sphingomyelinase in normal and Niemann-Pick fibroblasts.
Processing of human acid sphingomyelinase in normal and I-cell fibroblasts.
The p.L302P mutation in the lysosomal enzyme gene SMPD1 is a risk factor for Parkinson disease.
Mucopolysaccharidoses
Ito cells in lysosomal storage disorders. An ultrastructural study.
Multiple Myeloma
Decreased Activity of Blood Acid Sphingomyelinase in the Course of Multiple Myeloma.
Green tea polyphenol EGCG induces lipid raft clustering and apoptotic cell death by activating protein kinase C? and acid sphingomyelinase through 67-kDa laminin receptor in multiple myeloma cells.
The Transfer of Sphingomyelinase Contributes to Drug Resistance in Multiple Myeloma.
Multiple Sclerosis
A Robust Liposomal Platform for Direct Colorimetric Detection of Sphingomyelinase Enzyme and Inhibitors.
Acid sphingomyelinase deficiency enhances myelin repair after acute and chronic demyelination.
Acid sphingomyelinase: No potential as a biomarker for multiple sclerosis.
Cambinol, a novel inhibitor of neutral sphingomyelinase 2 shows neuroprotective properties.
Cytokines Stimulate the Release of Microvesicles from Myeloid Cells Independently from the P2X7 Receptor/Acid Sphingomyelinase Pathway.
Disruption of myelin sheaths in mouse brain in vitro and in vivo by staphylococcal alpha-toxin.
Gilenya (FTY720) inhibits acid sphingomyelinase by a mechanism similar to tricyclic antidepressants.
Oxidative stress kills human primary oligodendrocytes via neutral sphingomyelinase: implications for multiple sclerosis.
Pharmacological Inhibition of Acid Sphingomyelinase Ameliorates Experimental Autoimmune Encephalomyelitis.
Mumps
Comparative study of immune status to infectious agents in elderly patients with multiple myeloma, Waldenstrom's macroglobulinemia, and monoclonal gammopathy of undetermined significance.
Muscle Weakness
Diaphragm dysfunction caused by sphingomyelinase requires the p47(phox) subunit of NADPH oxidase.
Neutral sphingomyelinase 2 is required for cytokine-induced skeletal muscle calpain activation.
Sphingomyelinase depresses force and calcium sensitivity of the contractile apparatus in mouse diaphragm muscle fibers.
Sphingomyelinase stimulates oxidant signaling to weaken skeletal muscle and promote fatigue.
Myasthenia Gravis
[Binocular diplopia and ptosis due to snakebite (Agkistrodon blomhoffi "mamushi")--a case report]
Myelodysplastic Syndromes
Implications of sphingosine kinase 1 expression level for the cellular sphingolipid rheostat: relevance as a marker for daunorubicin sensitivity of leukemia cells.
Myocardial Infarction
Role of lipid rafts in ceramide and nitric oxide signaling in the ischemic and preconditioned hearts.
Small hairpin RNA and pharmacological targeting of neutral sphingomyelinase prevents diaphragm weakness in rats with heart failure and reduced ejection fraction.
Myositis
Effect of erythromycin on biological activities induced by clostridium perfringens alpha-toxin.
Myotoxicity
Identification of residues critical for toxicity in Clostridium perfringens phospholipase C, the key toxin in gas gangrene.
Nasal Polyps
Cellular responses to Staphylococcus aureus alpha-toxin in chronic rhinosinusitis with nasal polyps.
Nasopharyngeal Carcinoma
Identification of miRNA/mRNA-Negative Regulation Pairs in Nasopharyngeal Carcinoma.
Neoplasm Metastasis
A new twist to the emerging functions of ceramides in cancer: novel role for platelet acid sphingomyelinase in cancer metastasis.
A novel visible range FRET probe for monitoring acid sphingomyelinase activity in living cells.
Diagnosis and treatment of synchronous multiple primary lung cancer - a case report.
Early clinical diagnosis of synchronous multiple primary lung cancer.
Melanoma cell metastasis via P-selectin-mediated activation of acid sphingomyelinase in platelets.
MicroRNAs in Cancer: The 22nd Hiroshima Cancer Seminar/The 4th Japanese Association for RNA Interference Joint International Symposium, 30 August 2012, Grand Prince Hotel Hiroshima.
Neutral sphingomyelinase 2 (nSMase2)-dependent exosomal transfer of angiogenic microRNAs regulate cancer cell metastasis.
Neutral sphingomyelinase and breast cancer research.
Regulation of hematogenous tumor metastasis by acid sphingomyelinase.
Roles and regulation of neutral sphingomyelinase-2 in cellular and pathological processes.
Surgical Prognosis of Synchronous Multiple Primary Lung Cancer: Systematic Review and Meta-Analysis.
Surgical treatment of synchronous multiple primary lung cancers: a retrospective analysis of 122 patients.
[Research Progress in Distinguishing Methods of Simultaneous Multiple Primary Lung Cancer and Intrapulmonary Metastasis].
Neoplasms
"Cross talk" between the bioactive glycerolipids and sphingolipids in signal transduction.
'Patchiness' and basic cancer research: unravelling the proteases.
A neutral sphingomyelinase resides in sphingolipid-enriched microdomains and is inhibited by the caveolin-scaffolding domain: potential implications in tumour necrosis factor signalling.
A new twist to the emerging functions of ceramides in cancer: novel role for platelet acid sphingomyelinase in cancer metastasis.
A novel cisplatin mediated apoptosis pathway is associated with acid sphingomyelinase and FAS proapoptotic protein activation in ovarian cancer.
A novel cytoplasmic domain of the p55 tumor necrosis factor receptor initiates the neutral sphingomyelinase pathway.
A novel pathway for tumor necrosis factor-alpha and ceramide signaling involving sequential activation of tyrosine kinase, p21(ras), and phosphatidylinositol 3-kinase.
A staphylococcal alpha-toxin fragment. Its characterization and use for mapping biologically-active regions of alpha-toxin.
Abnormal methylation of seven genes and their associations with clinical characteristics in early stage non-small cell lung cancer.
Abnormal methylation status of FBXW10 and SMPD3, and associations with clinical characteristics in clear cell renal cell carcinoma.
Acid ceramidase but not acid sphingomyelinase is required for tumor necrosis factor-{alpha}-induced PGE2 production.
Acid sphingomyelinase and inhibition by phosphate ion: role of inhibition by phosphatidyl-myo-inositol 3,4,5-triphosphate in oligodendrocyte cell signaling.
Acid sphingomyelinase determines melanoma progression and metastatic behaviour via the microphtalmia-associated transcription factor signalling pathway.
Acid sphingomyelinase inhibition suppresses lipopolysaccharide-mediated release of inflammatory cytokines from macrophages and protects against disease pathology in dextran sulphate sodium-induced colitis in mice.
Acid sphingomyelinase involvement in tumor necrosis factor alpha-regulated vascular and steroid disruption during luteolysis in vivo.
Acid sphingomyelinase is not essential for the IL-1 and tumor necrosis factor receptor signaling pathway leading to NFkB activation.
Acid sphingomyelinase is required for cell surface presentation of Met receptor tyrosine kinase in cancer cells.
Acid sphingomyelinase overexpression enhances the antineoplastic effects of irradiation in vitro and in vivo.
Acid sphingomyelinase, a novel negative biomarker of ovarian cancer.
Acidic sphingomyelinase downregulates the liver-specific methionine adenosyltransferase 1A, contributing to tumor necrosis factor-induced lethal hepatitis.
Activation of endothelial nitric-oxide synthase by tumor necrosis factor-alpha: a novel pathway involving sequential activation of neutral sphingomyelinase, phosphatidylinositol-3' kinase, and Akt.
Activation of sphingosine kinase by tumor necrosis factor-alpha inhibits apoptosis in human endothelial cells.
Acute systemic inflammation up-regulates secretory sphingomyelinase in vivo: a possible link between inflammatory cytokines and atherogenesis.
Adenoviral transduction of human acid sphingomyelinase into neo-angiogenic endothelium radiosensitizes tumor cure.
Altered adipose and plasma sphingolipid metabolism in obesity: a potential mechanism for cardiovascular and metabolic risk.
Antiproliferative Effects of Thymoquinone in MCF-7 Breast and HepG2 Liver Cancer Cells: Possible Role of Ceramide and ER Stress.
Apolipoprotein C-I induces apoptosis in human aortic smooth muscle cells via recruiting neutral sphingomyelinase.
Apoptotic signaling through CD95 (Fas/Apo-1) activates an acidic sphingomyelinase.
Biochemical properties of mammalian neutral sphingomyelinase 2 and its role in sphingolipid metabolism.
Bioinformatic Exploration for Prognostic Significance of Sphingolipid Metabolism-Related Genes in Invasive Ductal Carcinoma Using the Cancer Genome Atlas Cohort.
Boswellic acid induces epigenetic alterations by modulating DNA methylation in colorectal cancer cells.
c-Jun is a downstream target for ceramide-activated protein phosphatase in A431 cells.
Caspase-8 and caspase-7 sequentially mediate proteolytic activation of acid sphingomyelinase in TNF-R1 receptosomes.
Cathepsin D links TNF-induced acid sphingomyelinase to Bid-mediated caspase-9 and -3 activation.
Caveolin-1 regulates the ASMase/ceramide-mediated radiation response of endothelial cells in the context of tumor-stroma interactions.
CD40 signals apoptosis through FAN-regulated activation of the sphingomyelin-ceramide pathway.
Ceramide accumulation is associated with increased apoptotic cell death in cultured fibroblasts of sphingolipid activator protein-deficient mouse but not in fibroblasts of patients with Farber disease.
Ceramide and cell death receptor clustering.
Ceramide and cyclic adenosine monophosphate (cAMP) induce cAMP response element binding protein phosphorylation via distinct signaling pathways while having opposite effects on myeloid cell survival.
Ceramide generated by acidic sphingomyelinase contributes to tumor necrosis factor-alpha-mediated apoptosis in human colon HT-29 cells through glycosphingolipids formation. Possible role of ganglioside GD3.
Ceramide plays a prominent role in MDA-7/IL-24-induced cancer-specific apoptosis.
Ceramide selectively inhibits early events in the response of human neutrophils to tumor necrosis factor.
Ceramide triggers p53-dependent apoptosis in genetically defined fibrosarcoma tumour cells.
Ceramide triggers Weibel-Palade body exocytosis.
Ceramide-induced cell death in malignant cells.
Ceramide-induced translocation of protein kinase C-delta and -epsilon to the cytosol. Implications in apoptosis.
Ceramide: an intracellular signal for apoptosis.
Ceramides that mediate apoptosis reduce glucose uptake and transporter affinity for glucose in human leukaemic cell lines but not in neutrophils.
Cervicovaginal Inflammatory Cytokines and Sphingomyelinase in Women With and Without Bacterial Vaginosis.
Characteristics and prognosis of synchronous multiple primary lung cancer after surgical treatment: A systematic review and meta-analysis of current evidence.
Characterization of the sphingomyelin content of isolated pancreatic islets. Evaluation of the role of sphingomyelin hydrolysis in the action of interleukin-1 to induce islet overproduction of nitric oxide.
Chemotherapeutic Agents-Induced Ceramide-Rich Platforms (CRPs) in Endothelial Cells and Their Modulation.
Cholesterol and sphingolipids in alcohol-induced liver injury.
Chronic Psychosocial Stress in Mice Is Associated With Increased Acid Sphingomyelinase Activity in Liver and Serum and With Hepatic C16:0-Ceramide Accumulation.
Comparative epigenomics of human and mouse mammary tumors.
Comparison of CD28-B7.1 and B7.2 functional interaction in resting human T cells: phosphatidylinositol 3-kinase association to CD28 and cytokine production.
Comprehensive genomic profiling aids in understanding the lesion origins of a patient with six synchronous invasive lung adenocarcinomas: a case study.
Crystal structure of mammalian acid sphingomyelinase.
Cytolysis mediated by ionophores and pore-forming agents: role of intracellular calcium in apoptosis.
De novo ceramide synthesis is responsible for the anti-tumor properties of camptothecin and doxorubicin in follicular thyroid carcinoma.
Decreasing the apoptotic threshold of tumor cells through protein kinase C inhibition and sphingomyelinase activation increases tumor killing by ionizing radiation.
Detection of alkaline sphingomyelinase activity in human stool: proposed role as a new diagnostic and prognostic marker of colorectal cancer.
Diagnosis and treatment of synchronous multiple primary lung cancer - a case report.
Differential effects of sphingomyelinase and cell-permeable ceramide analogs on proliferation of Swiss 3T3 fibroblasts.
Differential regulation of microRNA-15a by radiation affects angiogenesis and tumor growth via modulation of acid sphingomyelinase.
Differential regulation of sphingomyelinase and ceramidase activities by growth factors and cytokines. Implications for cellular proliferation and differentiation.
Distinct adapter proteins mediate acid versus neutral sphingomyelinase activation through the p55 receptor for tumor necrosis factor.
Divergent role of ceramide generated by exogenous sphingomyelinases on NF-kappa B activation and apoptosis in human colon HT-29 cells.
Drugs That Modify Cholesterol Metabolism Alter the p38/JNK-Mediated Targeted and Nontargeted Response to Alpha and Auger Radioimmunotherapy.
Early clinical diagnosis of synchronous multiple primary lung cancer.
Early events in the action of staphylococcal alpha-toxin on the plasma membrane of adrenocortical Y1 tumor cells.
Effect of erythromycin on biological activities induced by clostridium perfringens alpha-toxin.
Effects of staphylococcus alpha-toxin and streptolysin S on the oxidation of succinate by ascites tumour cells.
Effects of ursodeoxycholate and other bile salts on levels of rat intestinal alkaline sphingomyelinase: a potential implication in tumorigenesis.
Elevation of de novo ceramide synthesis in tumor masses and the role of microsomal dihydroceramide synthase.
Endothelial cell inflammatory responses to tumor necrosis factor alpha. Ceramide-dependent and -independent mitogen-activated protein kinase cascades.
Endothelial membrane remodeling is obligate for anti-angiogenic radiosensitization during tumor radiosurgery.
Endothelial nitric oxide synthase activation by tumor necrosis factor alpha through neutral sphingomyelinase 2, sphingosine kinase 1, and sphingosine 1 phosphate receptors: a novel pathway relevant to the pathophysiology of endothelium.
Enhanced acid sphingomyelinase activity drives immune evasion and tumor growth in non-small cell lung carcinoma.
Enhancement of Soft Tissue Sarcoma Response to Gemcitabine through Timed Administration of a Short-Acting Anti-Angiogenic Agent.
Essential role for acid sphingomyelinase-inhibited autophagy in melanoma response to cisplatin.
Evidence for involvement of mitogen-activated protein kinase, rather than stress-activated protein kinase, in potentiation of 1-beta-D-arabinofuranosylcytosine-induced apoptosis by interruption of protein kinase C signaling.
Fas-mediated apoptosis and sphingomyelinase signal transduction: the role of ceramide as a second messenger for apoptosis.
Fatty acid synthase causes drug resistance by inhibiting TNF-? and ceramide production.
Functional dichotomy of neutral and acidic sphingomyelinases in tumor necrosis factor signaling.
Functional inhibition of acid sphingomyelinase by Fluphenazine triggers hypoxia-specific tumor cell death.
Gentamicin Targets Acid Sphingomyelinase in Cancer: The Case of the Human Gastric Cancer NCI-N87 Cells.
Glutathione regulation of neutral sphingomyelinase in tumor necrosis factor-alpha-induced cell death.
Gynaecologic concerns for young women exposed to gonadotoxic chemotherapy.
Heat shock protein 70.1 (Hsp70.1) affects neuronal cell fate by regulating lysosomal acid sphingomyelinase.
HER2-positive breast cancer that resists therapeutic drugs and ionizing radiation releases sphingomyelin-based molecules to circulating blood serum.
High precision multi-genome scale reannotation of enzyme function by EFICAz.
Host acid sphingomyelinase regulates microvascular function not tumor immunity.
Human adipocyte differentiation and composition of disease-relevant lipids are regulated by miR-221-3p.
Human alkaline ceramidase 2 promotes the growth, invasion, and migration of hepatocellular carcinoma cells via sphingomyelin phosphodiesterase acid-like 3B.
Human hyperimmune globulin protects against the cytotoxic action of staphylococcal alpha-toxin in vitro and in vivo.
Identification and biochemical characterization of an acid sphingomyelinase-like protein from the bacterial plant pathogen Ralstonia solanacearum that hydrolyzes ATP to AMP but not sphingomyelin to ceramide.
Identification of aberrant forms of alkaline sphingomyelinase (NPP7) associated with human liver tumorigenesis.
Impaired cutaneous permeability barrier function, skin hydration, and sphingomyelinase activity in keratin 10 deficient mice.
Impaired neutral sphingomyelinase activation and cutaneous barrier repair in FAN-deficient mice.
Increased liver tumor formation in neutral sphingomyelinase-2 deficient mice.
Increased steroid hormone secretion in mouse Leydig tumor cells after induction of cholesterol translocation by sphingomyelin degradation.
Induction of apoptotic DNA damage and cell death by activation of the sphingomyelin pathway.
Induction of stress-activated protein kinases/c-Jun N-terminal kinases by the p55 tumour necrosis factor receptor does not require sphingomyelinases.
Induction of tissue factor production but not the upregulation of adhesion molecule expression by ceramide in human vascular endothelial cells.
Influence of Bax or Bcl-2 overexpression on the ceramide-dependent apoptotic pathway in glioma cells.
Inhibition of endothelial interleukin-8 production and neutrophil transmigration by Staphylococcus aureus beta-hemolysin.
Inhibition of neutral sphingomyelinase 2 promotes remyelination.
Inhibition of tumor necrosis factor-induced cell death in MCF7 by a novel inhibitor of neutral sphingomyelinase.
Interferons increase cell resistance to Staphylococcal alpha-toxin.
Interleukin-4 suppresses the enhancement of ceramide synthesis and cutaneous permeability barrier functions induced by tumor necrosis factor-alpha and interferon-gamma in human epidermis.
Involvement of Fas receptor and not tumor necrosis factor-alpha receptor in ultraviolet-induced activation of acid sphingomyelinase.
Ionizing radiations increase the activity of the cell surface glycohydrolases and the plasma membrane ceramide content.
It Takes a CAD to Kill a Tumor Cell with a LMP.
Kit signaling and negative regulation of daunorubicin-induced apoptosis: role of phospholipase Cgamma.
Management for Residual Ground-Glass Opacity Lesions After Resection of Main Tumor in Multifocal Lung Cancer: A Case Report and Literature Review.
Measurement of ceramide and sphingolipid metabolism in tumors: potential modulation of chemotherapy.
Membrane rafts-redox signalling pathway contributes to renal fibrosis via modulation of the renal tubular epithelial-mesenchymal transition.
Metabolic profiling-based data-mining for an effective chemical combination to induce apoptosis of cancer cells.
MicroRNAs in Cancer: The 22nd Hiroshima Cancer Seminar/The 4th Japanese Association for RNA Interference Joint International Symposium, 30 August 2012, Grand Prince Hotel Hiroshima.
Modification of LDL with human secretory phospholipase A(2) or sphingomyelinase promotes its arachidonic acid-releasing propensity.
Modulation of Acid Sphingomyelinase in Melanoma Reprogrammes the Tumour Immune Microenvironment.
Modulation of CYP3A4 expression by ceramide in human colon carcinoma HT-29 cells.
Molecular cloning, characterization, and expression of a novel human neutral sphingomyelinase.
Molecular modeling of human neutral sphingomyelinase provides insight into its molecular interactions.
Mutations in the neutral sphingomyelinase gene SMPD3 implicate the ceramide pathway in human leukemias.
Myelin contains neutral sphingomyelinase activity that is stimulated by tumor necrosis factor-alpha.
Neutral sphingomyelinase 2 (nSMase2)-dependent exosomal transfer of angiogenic microRNAs regulate cancer cell metastasis.
Neutral Sphingomyelinase 2 Heightens Anti-Melanoma Immune Responses and Anti-PD-1 Therapy Efficacy.
Neutral sphingomyelinase 2 inhibitors based on the 4-(1H-imidazol-2-yl)-2,6-dialkoxyphenol scaffold.
Neutral sphingomyelinase 2 modulates cytotoxic effects of protopanaxadiol on different human cancer cells.
Neutral sphingomyelinase action stimulates signal transduction of tumor necrosis factor-alpha in the synthesis of cholesteryl esters in human fibroblasts.
Neutral sphingomyelinase activation precedes NADPH oxidase-dependent damage in neurons exposed to the proinflammatory cytokine tumor necrosis factor-?.
Neutral sphingomyelinase and breast cancer research.
Neutral Sphingomyelinase-2 (nSMase2) is the Primary Neutral Sphingomyelinase Isoform Activated by Tumor Necrosis Factor-? in MCF-7 cells.
Neutral sphingomyelinase-3 is a DNA damage and nongenotoxic stress-regulated gene that is deregulated in human malignancies.
Neutral sphingomyelinase: past, present and future.
Neutral Sphingomyelinases in Cancer: Friend or Foe?
Next Generation Sequencing Reveals a Synchronous Trilateral Lung Adenocarcinoma Case with Distinct Driver Alterations of EGFR 19 Deletion or EGFR 20 Insertion or EZR-ROS1 Fusion.
Nitric Oxide Generated by Tumor-Associated Macrophages Is Responsible for Cancer Resistance to Cisplatin and Correlated With Syntaxin 4 and Acid Sphingomyelinase Inhibition.
Nitric oxide mediates natural polyphenol-induced Bcl-2 down-regulation and activation of cell death in metastatic B16 melanoma.
Novel tumor necrosis factor-responsive mammalian neutral sphingomyelinase-3 is a C-tail-anchored protein.
Off-target function of the Sonic hedgehog inhibitor cyclopamine in mediating apoptosis via nitric oxide-dependent neutral sphingomyelinase 2/ceramide induction.
Oligomerization of 3H-labelled staphylococcal alpha-toxin and fragments on adrenocortical Y1 tumour cells.
Omega-3 polyunsaturated fatty acids attenuate breast cancer growth through activation of a neutral sphingomyelinase-mediated pathway.
Optical and photoacoustic radiofrequency spectroscopic analysis for detecting red blood cell death.
Outcomes of Spatially Fractionated Radiotherapy (GRID) for Bulky Soft Tissue Sarcomas in a Large Animal Model.
Palmitate-activated astrocytes via serine palmitoyltransferase increase BACE1 in primary neurons by sphingomyelinases.
Pathological and Prognostic Indications of the mdig Gene in Human Lung Cancer.
Pazopanib radio-sensitization of human sarcoma tumors.
Primary synchronous small and non-small cell lung cancer in the same lung lobe: A case report.
Purification and characterization of a magnesium-dependent neutral sphingomyelinase from bovine brain.
Re-configuration of sphingolipid metabolism by oncogenic transformation.
Red blood cell clearance in inflammation.
Reduction in alkaline sphingomyelinase in colorectal tumorigenesis is not related to the APC gene mutation.
Regulated secretion of acid sphingomyelinase: implications for selectivity of ceramide formation.
Regulation of hematogenous tumor metastasis by acid sphingomyelinase.
Regulation of neutral sphingomyelinase-2 (nSMase2) by tumor necrosis factor-alpha involves protein kinase C-delta in lung epithelial cells.
Regulation of sphingomyelinases in cells of the oligodendrocyte lineage.
Regulatory T cells reduce endothelial neutral sphingomyelinase 2 to prevent T-cell migration into tumors.
Remodeling of cellular cytoskeleton by the acid sphingomyelinase/ceramide pathway.
Requirement of FADD for tumor necrosis factor-induced activation of acid sphingomyelinase.
Resveratrol-induced transcriptional up-regulation of ASMase (SMPD1) of human leukemia and cancer cells.
Riccardin D-N induces lysosomal membrane permeabilization by inhibiting acid sphingomyelinase and interfering with sphingomyelin metabolism in vivo.
Role for furin in tumor necrosis factor alpha-induced activation of the matrix metalloproteinase/sphingolipid mitogenic pathway.
Role for neutral sphingomyelinase-2 in tumor necrosis factor alpha-stimulated expression of vascular cell adhesion molecule-1 (VCAM) and intercellular adhesion molecule-1 (ICAM) in lung epithelial cells: p38 MAPK is an upstream regulator of nSMase2.
Role of Acid Sphingomyelinase and Ceramide in Mechano-Acoustic Enhancement of Tumor Radiation Responses.
Role of Acid Sphingomyelinase-Induced Signaling in Melanoma Cells for Hematogenous Tumor Metastasis.
Role of ceramide/sphingomyelin (SM) balance regulated through "SM cycle" in cancer.
Role of nuclear factor-kappaB activation in cytokine- and sphingomyelinase-stimulated inducible nitric oxide synthase gene expression in vascular smooth muscle cells.
Roles and regulation of neutral sphingomyelinase-2 in cellular and pathological processes.
Roles for tumor necrosis factor receptor p55 and sphingomyelinase in repairing the cutaneous permeability barrier.
Selected Clinical Features Fail to Predict Inflammatory Gene Expressions for TNF-?, TNFR1, NSMAF, Casp3 and IL-8 in Tendons of Patients with Rotator Cuff Tendinopathy.
SMPDL3B Predicts Poor Prognosis and Contributes to Development of Acute Myeloid Leukemia.
Sphingolipid Metabolism in Glioblastoma and Metastatic Brain Tumors: A Review of Sphingomyelinases and Sphingosine-1-Phosphate.
Sphingolipids in inflammatory hypoxia.
Sphingomyelin hydrolysis during apoptosis.
Sphingomyelin metabolism is a regulator of KRAS function.
Sphingomyelin phosphodiesterase 3 methylation and silencing in oral squamous cell carcinoma results in increased migration and invasion and altered stress response.
Sphingosine Kinase-1 Protects Multiple Myeloma from Apoptosis Driven by Cancer-Specific Inhibition of RTKs.
Sphingosine mediates the immediate negative inotropic effects of tumor necrosis factor-alpha in the adult mammalian cardiac myocyte.
Spinal ceramide modulates the development of morphine antinociceptive tolerance via peroxynitrite-mediated nitroxidative stress and neuroimmune activation.
Src Mediates Epigallocatechin-3-O-Gallate-Elicited Acid Sphingomyelinase Activation.
Staphylococcal toxins and protein A differentially induce cytotoxicity and release of tumor necrosis factor-alpha from human keratinocytes.
Staphylococcus aureus alpha-toxin induces apoptosis in peripheral blood mononuclear cells: role of endogenous tumour necrosis factor-alpha and the mitochondrial death pathway.
Staphylococcus aureus alpha-toxin inhibits CD8+ T cell-mediated killing of cancer cells in cutaneous T-cell lymphoma.
Stimulation of the ceramide pathway partially mimics lipopolysaccharide-induced responses in murine peritoneal macrophages.
Sunitinib and SU11652 Inhibit Acid Sphingomyelinase, Destabilize Lysosomes, and Inhibit Multidrug Resistance.
Surgical Prognosis of Synchronous Multiple Primary Lung Cancer: Systematic Review and Meta-Analysis.
Survival and prognostic factors in surgically resected synchronous multiple primary lung cancers.
SUVmax and Tumor Size Predict Surgical Outcome of Synchronous Multiple Primary Lung Cancers.
SUVmax Ratio on PET/CT May Differentiate Between Lung Metastases and Synchronous Multiple Primary Lung Cancer.
Synovial fibroblasts and the sphingomyelinase pathway: sphingomyelin turnover and ceramide generation are not signaling mechanisms for the actions of tumor necrosis factor-alpha.
Syntaxin 4 is required for acid sphingomyelinase activity and apoptotic function.
Targeting acid sphingomyelinase with anti-angiogenic chemotherapy.
The Effects of ASMase Mediated Endothelial Cell Apoptosis in Multiple Hypofractionated Irradiations in CT26 Tumor Bearing Mice.
The inhibition of lipopolysaccharide-induced tumor necrosis factor-alpha and nitric oxide production by Clostridium perfringens alpha-toxin and its relation to alpha-toxin-induced intracellular ceramide generation.
The juxtamembrane linker in neutral sphingomyelinase-2 functions as an intramolecular allosteric switch that activates the enzyme.
The Roles of Neutral Sphingomyelinases in Neurological Pathologies.
TNF receptor death domain-associated proteins TRADD and FADD signal activation of acid sphingomyelinase.
TNF-alpha-induced endothelium-independent vasodilation: a role for phospholipase A2-dependent ceramide signaling.
Toxicity of oxidized phosphatidylcholines in cultured human melanoma cells.
Transformation-associated changes in sphingolipid metabolism sensitize cells to lysosomal cell death induced by inhibitors of Acid sphingomyelinase.
Tumor cell resistance to apoptosis due to a defect in the activation of sphingomyelinase and the 24 kDa apoptotic protease (AP24).
Tumor necrosis factor (TNF)-alpha activates c-raf-1 kinase via the p55 TNF receptor engaging neutral sphingomyelinase.
Tumor necrosis factor (TNF)-alpha inhibits insulin signaling through stimulation of the p55 TNF receptor and activation of sphingomyelinase.
Tumor necrosis factor alpha increases neuronal vulnerability to excitotoxic necrosis by inducing expression of the AMPA-glutamate receptor subunit GluR1 via an acid sphingomyelinase- and NF-kappaB-dependent mechanism.
Tumor necrosis factor alpha stimulates sphingomyelinase through the 55 kDa receptor in HL-60 cells.
Tumor necrosis factor-alpha stimulates the maturation of sterol regulatory element binding protein-1 in human hepatocytes through the action of neutral sphingomyelinase.
Tumor necrosis factor-alpha, sphingomyelinase and ceramides activate tyrosine kinase, p21Ras and phosphatidylinositol 3-kinase: implications for glucose transport and insulin resistance.
Tumor necrosis factor-alpha, sphingomyelinase, and ceramide inhibit store-operated calcium entry in thyroid FRTL-5 cells.
Tumor necrosis factor-alpha-induced neutral sphingomyelinase-2 modulates synaptic plasticity by controlling the membrane insertion of NMDA receptors.
Tumor response to radiotherapy regulated by endothelial cell apoptosis.
Unexpected encounters: high prevalence of synchronous primary lung cancers in a tertiary academic center.
Use of acid sphingomyelinase for cancer therapy.
[Analysis on the Prognostic and Survival Factors of Synchronous Multiple Primary Lung Cancer].
[Ceramide: a lipid mediator of apoptotic signal transduction]
[Changes in sphingomyelinase activity, tumor necrosis factor alpha level, and lipid peroxidation rate in the course of development of cholestatic liver injury]
[Changes in the activity of neutral and acidic isoforms of sphingomyelinase in hepatoma-22, regenerating and ischemic liver]
[Concordant point mutation of ETS-related gene (ERG) in tumor tissues from a synchronous multiple primary lung cancer: A case report].
[Functional state of a sphingomyeline cycle and free radical lipid oxidation activity of a rat's liver during different phases of starvation].
[Relationship of tumor necrosis factor alpha expression with activation of sphingomyelinase and lipid peroxidation after removal of cholestatic factor]
[Synchronous Multiple Primary Lung Cancer Dignosed ?by Different Phenotype-genotype: A Case Report and Literature Review].
[The effect of tumor necrosis factor on the free sphingosine level and sphingomyelinase in murine liver cells and nuclei]
[TNF-? regulates the proliferation of human breast cancer cells via regulation of ceramide content].
Nephritis
Altered sphingomyelinase and ceramide expression in the setting of ischemic and nephrotoxic acute renal failure.
Nephrotic Syndrome
Podocyte sphingomyelin phosphodiesterase acid-like 3b decreases among children with idiopathic nephrotic syndrome.
Urinary excretion of sphingomyelinase phosphodiesterase acid-like 3b in children with intractable nephrotic syndrome.
Nervous System Diseases
A novel and potent brain penetrant inhibitor of extracellular vesicle release.
Bilateral Cystic Bronchiectasis as Novel Phenotype of Niemann-Pick Disease Type B Successfully Treated With Double Lung Transplantation.
Interactions between neural membrane glycerophospholipid and sphingolipid mediators: a recipe for neural cell survival or suicide.
Neutral sphingomyelinase 2 inhibitors based on the 4-(1H-imidazol-2-yl)-2,6-dialkoxyphenol scaffold.
The Roles of Neutral Sphingomyelinases in Neurological Pathologies.
Neurilemmoma
The ATP-binding cassette transporter-2 (ABCA2) regulates esterification of plasma membrane cholesterol by modulation of sphingolipid metabolism.
Neuroblastoma
Antidepressant-induced lipidosis with special reference to tricyclic compounds.
Catabolism of exogenous and endogenous sphingomyelin and phosphatidylcholine by homogenates and subcellular fractions of cultured neuroblastoma cells. Effects of anesthetics.
Evidence that neutral sphingomyelinase of cultured murine neuroblastoma cells is oriented externally on the plasma membrane.
Ionizing radiations increase the activity of the cell surface glycohydrolases and the plasma membrane ceramide content.
Localization of neutral magnesium-stimulated sphingomyelinase in plasma membrane of cultured neuroblastoma cells.
Neutral sphingomyelinase activation precedes NADPH oxidase-dependent damage in neurons exposed to the proinflammatory cytokine tumor necrosis factor-?.
Sphingomyelinase metabolites control survival and apoptotic death in SH-SY5Y neuroblastoma cells.
The role of gangliosides in fenretinide-induced apoptosis of neuroblastoma.
Tricyclic antidepressants induce sphingomyelinase deficiency in fibroblast and neuroblastoma cell cultures.
Neurodegenerative Diseases
Acid Sphingomyelinase Inhibition Mitigates Histopathologic and Behavioral Changes in a Murine Model of Traumatic Brain Injury.
Alterations of myelin-specific proteins and sphingolipids characterize the brains of acid sphingomyelinase-deficient mice, an animal model of Niemann-Pick disease type A.
Neutral sphingomyelinase increases and delocalizes in the absence of Toll-Like Receptor 4: A new insight for MPTP neurotoxicity.
Pharmacological inhibition of nSMase2 reduces brain exosome release and ?-synuclein pathology in a Parkinson's disease model.
Potential therapeutic target for aging and age-related neurodegenerative diseases: the role of acid sphingomyelinase.
Promising neuroprotective effects of ?-caryophyllene against LPS-induced oligodendrocyte toxicity: A mechanistic study.
Neuroinflammatory Diseases
Acid sphingomyelinase activity triggers microparticle release from glial cells.
Inhibition of extracellular vesicle pathway using neutral sphingomyelinase inhibitors as a neuroprotective treatment for brain injury.
Neutral Sphingomyelinase Behaviour in Hippocampus Neuroinflammation of MPTP-Induced Mouse Model of Parkinson's Disease and in Embryonic Hippocampal Cells.
Neutral Sphingomyelinase Inhibition Alleviates LPS-Induced Microglia Activation and Neuroinflammation after Experimental Traumatic Brain Injury.
Neurologic Manifestations
Exosomes mediate Zika virus transmission through SMPD3 neutral Sphingomyelinase in cortical neurons.
Neuronal Ceroid-Lipofuscinoses
Bi-functional IgG-lysosomal enzyme fusion proteins for brain drug delivery.
Nevus
BETA-GALACTOSYLCERAMIDASE PROMOTES MELANOMA GROWTH VIA MODULATION OF CERAMIDE METABOLISM.
Niemann-Pick Disease, Type A
?-Tocopherol Effect on Endocytosis and Its Combination with Enzyme Replacement Therapy for Lysosomal Disorders: A New Type of Drug Interaction?
A family with visceral course of Niemann-Pick disease, macular halo syndrome and low sphingomyelin degradation rate.
A new variant of sphingomyelinase deficiency (Niemann-Pick): visceromegaly, minimal neurological lesions and low in vivo degradation rate of sphingomyelin.
A novel association between angiokeratoma corporis diffusum and acid sphingomyelinase deficiency.
AAV8-mediated hepatic expression of acid sphingomyelinase corrects the metabolic defect in the visceral organs of a mouse model of Niemann-Pick disease.
Acid sphingomyelinase deficiency (Niemann?Pick disease Type B) as an inflammatory disease.
Acid Sphingomyelinase Deficiency Ameliorates Farber Disease.
Acid sphingomyelinase deficiency attenuates bleomycin-induced lung inflammation and fibrosis in mice.
Acid sphingomyelinase deficiency contributes to resistance of scleroderma fibroblasts to Fas-mediated apoptosis.
Acid sphingomyelinase deficiency does not protect from graft-versus-host disease in transplant recipients with Niemann-Pick disease.
Acid sphingomyelinase deficiency enhances myelin repair after acute and chronic demyelination.
Acid sphingomyelinase deficiency exacerbates LPS-induced experimental periodontitis.
Acid sphingomyelinase deficiency in Beckwith Wiedemann syndrome.
Acid sphingomyelinase deficiency in Western diet-fed mice protects against adipocyte hypertrophy and diet-induced liver steatosis.
Acid sphingomyelinase deficiency increases susceptibility to fatal alphavirus encephalomyelitis.
Acid Sphingomyelinase Deficiency Prevents Diet-induced Hepatic Triacylglycerol Accumulation and Hyperglycemia in Mice.
Acid sphingomyelinase deficiency protects from cisplatin-induced gastrointestinal damage.
Acid sphingomyelinase deficiency protects mitochondria and improves function recovery after brain injury.
Acid sphingomyelinase deficiency. Phenotype variability with prevalence of intermediate phenotype in a series of twenty-five Czech and Slovak patients. A multi-approach study.
Acid sphingomyelinase deficiency: Cardiac dysfunction and characteristic findings of the coronary arteries.
Acid sphingomyelinase deficiency: prevalence and characterization of an intermediate phenotype of Niemann-Pick disease.
Acid Sphingomyelinase Inhibition Prevents Development of Sepsis Sequelae in the Murine Liver.
Acid sphingomyelinase possesses a domain homologous to its activator proteins: saposins B and D.
Adrenal changes in Niemann-Pick disease: differences between sphingomyelinase deficiency and type C.
Adult Niemann-Pick disease masquerading as sea blue histiocyte syndrome: report of a case confirmed by lipid analysis and enzyme assays.
Adult sphingomyelinase deficiency: report of 2 patients who initially presented with psychiatric disorders.
Altered Lipid Metabolism in Brain Injury and Disorders.
Altered lipid metabolism in brain injury and disorders.
Altered Macrophage Function Associated with Crystalline Lung Inflammation in Acid Sphingomyelinase Deficiency.
An animal model of human acid sphingomyelinase deficiency (Niemann-Pick disease) and the study of its enzyme replacement (the Japan Society of Human Genetics award lecture).
An Early-Onset Neuronopathic Form of Acid Sphingomyelinase Deficiency: A SMPD1 p.C133Y Mutation in the Saposin Domain of Acid Sphingomyelinase.
Application of a glycinated bile acid biomarker for diagnosis and assessment of response to treatment in Niemann-pick disease type C1.
Autopsy pathology of infantile neurovisceral ASMD (Niemann-Pick Disease type A): Clinicopathologic correlations of a case report.
Biochemical and imaging parameters in acid sphingomyelinase deficiency: Potential utility as biomarkers.
Burden of Illness in Acid Sphingomyelinase Deficiency: A Retrospective Chart Review of 100 Patients.
Case Report of Gastrointestinal Bleeding in an Adult with Chronic Visceral Acid Sphingomyelinase Deficiency.
Cause of death in patients with chronic visceral and chronic neurovisceral acid sphingomyelinase deficiency (Niemann-Pick disease type B and B variant): Literature review and report of new cases.
Chronic Niemann-Pick disease with sphingomyelinase deficiency in two brothers with mental retardation.
Chronic visceral acid sphingomyelinase deficiency (Niemann-Pick disease type B) in 16 Polish patients: long-term follow-up.
Clathrin-mediated endocytosis is impaired in type A-B Niemann-Pick disease model cells and can be restored by ICAM-1-mediated enzyme replacement.
Clearance of Hepatic Sphingomyelin by Olipudase Alfa Is Associated With Improvement in Lipid Profiles in Acid Sphingomyelinase Deficiency.
Clinical relevance of endpoints in clinical trials for acid sphingomyelinase deficiency enzyme replacement therapy.
Combined Emphysema and Interstitial Lung Disease as a Rare Presentation of Pulmonary Involvement in a Patient with Chronic Visceral Acid Sphingomyelinase Deficiency (Niemann-Pick Disease Type B).
Complex kinetics of bis(4-methylumbelliferyl)phosphate and hexadecanoyl(nitrophenyl)phosphorylcholine hydrolysis by purified sphingomyelinase in the presence of Triton X-100.
Consensus recommendation for a diagnostic guideline for acid sphingomyelinase deficiency.
Correction of sphingomyelinase deficiency in Niemann-Pick type C fibroblasts by removal of lipoprotein fraction from culture media.
Corrigendum to "Cause of death in patients with chronic visceral and chronic neurovisceral acid sphingomyelinase deficiency (Niemann-Pick disease type B and B variant): Literature review and report of new cases" [Mol. Genet. Metab. 118 (2016) 206-213].
Critical assessment of chitotriosidase analysis in the rational laboratory diagnosis of children with Gaucher disease and Niemann-Pick disease type A/B and C.
Deficiency of phospholipase C acting on phosphatidylglycerol in Niemann-Pick disease.
Deficiency of taurocholate-dependent phospholipase C acting on phosphatidylcholine in Niemann-Pick disease.
Deposition of lipopigment--a new feature of human splenic sinus endothelium (SSE). Ultrastructural and histochemical study.
Disease manifestations and burden of illness in patients with acid sphingomyelinase deficiency (ASMD).
Dual diagnosis of Ochoa syndrome and Niemann-Pick disease type B in a consanguineous family.
Effect of dimethylsulfoxide on sphingomyelinase activity and cholesterol metabolism in Niemann-Pick type C fibroblasts.
Effect of host cell lipid metabolism on alphavirus replication, virion morphogenesis, and infectivity.
Effects of acid sphingomyelinase deficiency on male germ cell development and programmed cell death.
Effects of dimethylsulfoxide on sphingomyelinase in cultured human fibroblasts and correction of sphingomyelinase deficiency in fibroblasts from Niemann-Pick patients.
Enhanced Delivery and Effects of Acid Sphingomyelinase by ICAM-1-Targeted Nanocarriers in Type B Niemann-Pick Disease Mice.
Functional characterization of novel variants in SMPD1 in Indian patients with acid sphingomyelinase deficiency.
Gelastic cataplexy in Niemann-Pick disease group C and related variants without generalized sphingomyelinase deficiency.
Homozygous pArg610del Mutation Unusually Associated With Severe Delay of Growth in 2 Acid Sphingomyelinase Deficiency-affected Sibs.
Identification and expression of a common missense mutation (L302P) in the acid sphingomyelinase gene of Ashkenazi Jewish type A Niemann-Pick disease patients.
Identification and expression of a missense mutation (Y446C) in the acid sphingomyelinase gene from a Japanese patient with type A Niemann-Pick disease.
Identification of a distinct mutation spectrum in the SMPD1 gene of Chinese patients with acid sphingomyelinase-deficient Niemann-Pick disease.
Inhibition of fatty acid amide hydrolase prevents pathology in neurovisceral acid sphingomyelinase deficiency by rescuing defective endocannabinoid signaling.
Interstitial lung disease in lysosomal storage disorders.
Ito cells in lysosomal storage disorders. An ultrastructural study.
Limited benefits of presymptomatic cord blood transplantation in neurovisceral acid sphingomyelinase deficiency (ASMD) intermediate type.
Lipid abnormalities in children with types A and B Niemann Pick disease.
Lipid abnormalities in foam cell reticulosis of mice, an analogue of human sphingomyelin lipidosis.
Liver and skin histopathology in adults with acid sphingomyelinase deficiency (Niemann-Pick disease type B).
Long-term efficacy of olipudase alfa in adults with acid sphingomyelinase deficiency (ASMD): Further clearance of hepatic sphingomyelin is associated with additional improvements in pro- and anti-atherogenic lipid profiles after 42 months of treatment.
Lysosomal involvement in cellular turnover of plasma membrane sphingomyelin.
Macula halo syndrome. Variant of Niemann-Pick disease.
Mitochondrial biogenesis is transcriptionally repressed in lysosomal lipid storage diseases.
Molecular analysis of the acid sphingomyelinase deficiency in a family with an intermediate form of Niemann-Pick disease.
Molecular basis of acid sphingomyelinase deficiency in a patient with Niemann-Pick disease type A.
MRI in an unusually protracted neuronopathic variant of acid sphingomyelinase deficiency.
New findings in pathology of storage.
Niemann-Pick disease and juvenile xanthogranuloma. Are they related?
Niemann-Pick disease type A and B are clinically but also enzymatically heterogeneous: pitfall in the laboratory diagnosis of sphingomyelinase deficiency associated with the mutation Q292 K.
Niemann-Pick disease type C. Pathological, histochemical, ultrastructural and biochemical studies.
Niemann-Pick Disease versus acid sphingomyelinase deficiency.
Niemann-Pick disease.
Niemann-Pick type C disease: Novel NPC1 mutations and characterization of the concomitant acid sphingomyelinase deficiency.
Niemann-Pick type C disease: The atypical sphingolipidosis.
Nonclinical safety assessment of recombinant human acid sphingomyelinase (rhASM) for the treatment of acid sphingomyelinase deficiency:the utility of animal models of disease in the toxicological evaluation of potential therapeutics.
Novel mutations in the SMPD1 gene in Jordanian children with Acid sphingomyelinase deficiency (Niemann-Pick types A and B).
Ocular manifestations of adult Niemann-Pick disease: a case report.
Ocular pathology of infantile Niemann-Pick disease. Study of fetus of 23 weeks' gestation.
Olipudase alfa for treatment of acid sphingomyelinase deficiency (ASMD): safety and efficacy in adults treated for 30 months.
One-year results of a clinical trial of olipudase alfa enzyme replacement therapy in pediatric patients with acid sphingomyelinase deficiency.
Optimization of a histopathological biomarker for sphingomyelin accumulation in Acid sphingomyelinase deficiency.
Oral, dental, and craniofacial features in chronic acid sphingomyelinase deficiency.
Partial sphingomyelinase deficiency with sea-blue histiocytosis and neurovisceral dysfunction.
Pathogenesis of one variant of sea-blue histiocytosis.
Periodontal condition and treatment in a patient with rare systemic condition: A case report for acid sphingomyelinase deficiency.
Peripheral neuropathy in type A Niemann-Pick disease. A morphological study.
Phenotypic and genetic heterogeneity in Niemann-Pick disease type C: current knowledge and practical implications.
Plasma lyso-sphingomyelin levels are positively associated with clinical severity in acid sphingomyelinase deficiency.
Polyneuropathy in feline Niemann-Pick disease.
Prospective study of the natural history of chronic acid sphingomyelinase deficiency in children and adults: eleven years of observation.
Quantitation of plasmatic lysosphingomyelin and lysosphingomyelin-509 for differential screening of Niemann-Pick A/B and C diseases.
Quantitative Systems Pharmacology Modeling of Acid Sphingomyelinase Deficiency and the Enzyme Replacement Therapy Olipudase Alfa Is an Innovative Tool for Linking Pathophysiology and Pharmacology.
Radiation-induced apoptosis of endothelial cells in the murine central nervous system: protection by fibroblast growth factor and sphingomyelinase deficiency.
Rapid screening for lipid storage disorders using biochemical markers. Expert center data and review of the literature.
Recommendations for clinical monitoring of patients with acid sphingomyelinase deficiency (ASMD).
Reconstruction of the Cytokine Signaling in Lysosomal Storage Diseases by Literature Mining and Network Analysis.
Regulation of Chlamydomonas flagella and ependymal cell motile cilia by ceramide-mediated translocation of GSK3.
Reliable Assay of Acid Sphingomyelinase Deficiency with the Mutation Q292K by Tandem Mass Spectrometry.
Respiratory impairment in Niemann-Pick B disease: Two case reports and review for the pulmonologist.
Serum Zn(2+)-stimulated sphingomyelinase deficiency in type B Niemann-Pick disease.
Seven novel mutations of the SMPD1 gene in four Chinese patients with Niemann-Pick disease type A and prenatal diagnosis for four fetuses.
Signaling in and regulation of ionizing radiation-induced apoptosis in endothelial cells.
Skeletal manifestations in pediatric and adult patients with Niemann Pick disease type B.
Sphingomyelin lipidosis (Niemann-Pick disease) in a juvenile raccoon (Procyon lotor).
Sphingomyelinase activities of various human tissues in control subjects and in Niemann-Pick disease - development and evaluation of a microprocedure.
Sphingomyelinase deficiency (Niemann-Pick disease) in a Hereford calf.
Splenic histiocytosis in idiopathic thrombocytopenic purpura: a relative sphingomyelinase deficiency?
Structural and functional analysis of the ASM p.Ala359Asp mutant that causes acid sphingomyelinase deficiency.
Subclinical course of adult visceral Niemann-Pick type C1 disease. A rare or underdiagnosed disorder?
Successful within-patient dose escalation of olipudase alfa in acid sphingomyelinase deficiency.
Synthesis of pyrenesulfonylamido-sphingomyelin and its use as substrate for determining sphingomyelinase activity and diagnosing Niemann-Pick disease.
The IL-1 receptor signaling pathway.
The impact of biomarkers analysis in the diagnosis of Niemann-Pick C disease and acid sphingomyelinase deficiency.
The natural history of type B Niemann-Pick disease: results from a 10-year longitudinal study.
Treatment of sphingomyelinase deficiency by repeated implantations of amniotic epithelial cells.
Tricyclic antidepressants induce sphingomyelinase deficiency in fibroblast and neuroblastoma cell cultures.
Twenty- five years of biochemical diagnosis of Gaucher disease: the Egyptian experience.
Two new mutations in the acid sphingomyelinase gene causing type a Niemann-pick disease: N389T and R441X.
Two novel mutations in patients with atypical phenotypes of acid sphingomyelinase deficiency.
Two Siblings with Niemann-Pick Disease (NPD) Type B: Clinical Findings and Novel Mutations of the Acid Sphingomyelinase Gene.
Type A Niemann-Pick disease: a frameshift mutation in the acid sphingomyelinase gene (fsP330) occurs in Ashkenazi Jewish patients.
Uptake and metabolism of radioactively labeled sphingomyelin in cultured skin fibroblasts from controls and patients with Niemann-Pick disease and other lysosomal storage diseases.
[Acid sphingomyelinase deficiency (Niemann-Pick disease type B) in adulthood: A retrospective multicentric study of 28 adult cases].
[Acid sphingomyelinase deficiency and spleen trauma: Splenectomy or not splenectomy?]
[Activators for sphingohydrolases and the nature of the sphingomyelinase deficiency in Niemann-Pick disease types A, B and C (author's transl)]
[Lipidosis with vertical gaze palsy, macular degeneration, and sphingomyelinase deficiency]
[Lysosomal sphingomyelinase deficiency: spectrum of phenotypes in Czech and Slovak patients]
[Niemann-Pick disease revealed late by miliary tuberculosis: diagnosis by leukocyte determination of sphingomyelinase deficiency]
[Perhexiline maleate induces sphingomyelinase deficiency in man]
[Sea-blue histiocyte syndrome (author's transl)]
[Sphingomyelinase deficiency (Niemann-Pick) type A. A new variant with an unusually prolonged course]
[Study of thesaurismosis induced by perhexiline maleate. Confirmation of experimental data]
Niemann-Pick Disease, Type B
A novel missense mutation of the SMPD1 gene in a Taiwanese patient with type B Niemann-Pick disease.
A novel SMPD1 mutation in two Chinese sibling patients with type B Niemann-Pick disease.
Acid sphingomyelinase overexpression enhances the antineoplastic effects of irradiation in vitro and in vivo.
Delivery of acid sphingomyelinase in normal and niemann-pick disease mice using intercellular adhesion molecule-1-targeted polymer nanocarriers.
Elevated sphingomyelinase and hypercytokinemia in hemophagocytic lymphohistiocytosis.
Enhanced Delivery and Effects of Acid Sphingomyelinase by ICAM-1-Targeted Nanocarriers in Type B Niemann-Pick Disease Mice.
ICAM-1 targeting, intracellular trafficking, and functional activity of polymer nanocarriers coated with a fibrinogen-derived peptide for lysosomal enzyme replacement.
Identification of a missense mutation (S436R) in the acid sphingomyelinase gene from a Japanese patient with type B Niemann-Pick disease.
Ophthalmologic manifestations of type B Niemann-Pick diseases.
R542X mutation in SMPD1 gene: genetically novel mutation with phenotypic features intermediate between type A and type B Niemann-Pick disease.
Recombinant human acid sphingomyelinase as an adjuvant to sorafenib treatment of experimental liver cancer.
Reduced cellular cholesterol efflux and low plasma high-density lipoprotein cholesterol in a patient with type B Niemann-Pick disease because of a novel SMPD-1 mutation.
Serum Zn(2+)-stimulated sphingomyelinase deficiency in type B Niemann-Pick disease.
The natural history of type B Niemann-Pick disease: results from a 10-year longitudinal study.
Uptake and metabolism of radioactively labeled sphingomyelin in cultured skin fibroblasts from controls and patients with Niemann-Pick disease and other lysosomal storage diseases.
Niemann-Pick Disease, Type C
A progressive neurologic disorder with supranuclear vertical gaze paresis and distinctive bone marrow cells.
Biochemical and ultrastructural studies on an Epstein-Barr virus-transformed lymphoid cell line from a Niemann-Pick disease type C patient.
Chromatofocusing of skin fibroblast sphingomyelinase: alterations in Niemann-Pick disease type C shared by GM1-gangliosidosis.
Critical assessment of chitotriosidase analysis in the rational laboratory diagnosis of children with Gaucher disease and Niemann-Pick disease type A/B and C.
Effects of dimethylsulfoxide on sphingomyelinase activities in normal and Niemann-Pick type A, B and C fibroblasts.
Isoenzymes of sphingomyelinase and the genetic defect in Niemann-Pick disease, type C.
Juvenile dystonic lipidosis (variant of Niemann-Pick disease type C).
Macrophage uptake of oxidized LDL inhibits lysosomal sphingomyelinase, thus causing the accumulation of unesterified cholesterol-sphingomyelin-rich particles in the lysosomes. A possible role for 7-Ketocholesterol.
Morphological diagnosis of Niemann-Pick disease type C by skin and conjunctival biopsies.
Niemann-Pick disease type C: an update.
Niemann-Pick disease type D: lipid analyses and studies on sphingomyelinases.
Niemann-Pick disease, Type C: evidence for the deficiency of an activating factor stimulating sphingomyelin and glucocerebroside degradation.
Niemann-Pick disease: coupling and uncoupling of inhibited sphingomyelinase activity and exogenous cholesterol esterification in fibroblasts by ionophore treatment.
Niemann-Pick diseases.
Niemann-Pick type C disease: Novel NPC1 mutations and characterization of the concomitant acid sphingomyelinase deficiency.
Posttranslational regulation of acid sphingomyelinase in niemann-pick type C1 fibroblasts and free cholesterol-enriched chinese hamster ovary cells.
Prenatal diagnosis of Niemann-Pick disease type C.
Quantitation of plasmatic lysosphingomyelin and lysosphingomyelin-509 for differential screening of Niemann-Pick A/B and C diseases.
Rapid screening for lipid storage disorders using biochemical markers. Expert center data and review of the literature.
Resolution of tissue sphingomyelinase isoelectric profile in multiple components is extraction-dependent: evidence for a component defect in Niemann-Pick disease type C is spurious.
Sphingomyelin storage in a patient with myoclonus epilepsy as a main clinical symptom -- a varient in Niemann-Pick disease type C.
Sphingomyelinase activities of various human tissues in control subjects and in Niemann-Pick disease - development and evaluation of a microprocedure.
Sphingomyelinase and nonspecific phosphodiesterase activities in Epstein-Barr virus-transformed lymphoid cell lines from Niemann-Pick disease A, B and C.
Sphingomyelinase defect in Niemann-Pick disease, type C, fibroblasts.
Sphingomyelinase in cultured skin fibroblasts from normal and Niemann-Pick type C patients.
Subclinical course of adult visceral Niemann-Pick type C1 disease. A rare or underdiagnosed disorder?
The impact of biomarkers analysis in the diagnosis of Niemann-Pick C disease and acid sphingomyelinase deficiency.
[Niemann-Pick disease type C (subacute neurovisceral lipidosis). Problems of altered sphingomyelinase activity in the brain (author's transl)]
Niemann-Pick Diseases
?-Tocopherol Effect on Endocytosis and Its Combination with Enzyme Replacement Therapy for Lysosomal Disorders: A New Type of Drug Interaction?
A family with visceral course of Niemann-Pick disease, macular halo syndrome and low sphingomyelin degradation rate.
A fluorescence-based, high-performance liquid chromatographic assay to determine acid sphingomyelinase activity and diagnose types A and B Niemann-Pick disease.
A fluorescence-based, high-throughput sphingomyelin assay for the analysis of Niemann-Pick disease and other disorders of sphingomyelin metabolism.
A fluorometric determination of sphingomyelinase by use of fluorescent derivatives of sphingomyelin, and its application to diagnosis of Niemann-Pick disease.
A micromethod for sphingomyelinase assay using a chromogenic artificial substrate. Its use in the diagnosis of Niemann-Pick disease.
A micromethod for sphingomyelinase assay using a chromogenic artificial substrate: its use in the diagnosis of Niemann-Pick disease.
A neutral sphingomyelinase resides in sphingolipid-enriched microdomains and is inhibited by the caveolin-scaffolding domain: potential implications in tumour necrosis factor signalling.
A new form of Niemann-Pick disease characterised by temperature-labile sphingomyelinase.
A novel missense mutation of the SMPD1 gene in a Taiwanese patient with type B Niemann-Pick disease.
A novel missense SMPD1 gene mutation, T460P, and clinical findings in a patient with Niemann-Pick disease type B presenting to a lipid disorders clinic.
A novel SMPD1 mutation in two Chinese sibling patients with type B Niemann-Pick disease.
A practical chromogenic procedure for the detection of homozygotes and heterozygous carriers of Niemann-Pick disease.
A profound deficiency of (CH3-14C)choline sphingomyelin-cleaving enzyme in Niemann-Pick disease type B.
A progressive neurologic disorder with supranuclear vertical gaze paresis and distinctive bone marrow cells.
AAV8-mediated hepatic expression of acid sphingomyelinase corrects the metabolic defect in the visceral organs of a mouse model of Niemann-Pick disease.
Accurate differentiation of neuronopathic and nonneuronopathic forms of Niemann-Pick disease by evaluation of the effective residual lysosomal sphingomyelinase activity in intact cells.
Acid sphingomyelinase (Asm) deficiency patients in The Netherlands and Belgium: Disease spectrum and natural course in attenuated patients.
Acid sphingomyelinase (aSMase) deficiency leads to abnormal microglia behavior and disturbed retinal function.
Acid sphingomyelinase deficiency does not protect from graft-versus-host disease in transplant recipients with Niemann-Pick disease.
Acid sphingomyelinase deficiency exacerbates LPS-induced experimental periodontitis.
Acid sphingomyelinase deficiency. Phenotype variability with prevalence of intermediate phenotype in a series of twenty-five Czech and Slovak patients. A multi-approach study.
Acid sphingomyelinase deficiency: prevalence and characterization of an intermediate phenotype of Niemann-Pick disease.
Acid sphingomyelinase deficient mice: a model of types A and B Niemann-Pick disease.
Acid sphingomyelinase is not essential for the IL-1 and tumor necrosis factor receptor signaling pathway leading to NFkB activation.
Acid sphingomyelinase overexpression enhances the antineoplastic effects of irradiation in vitro and in vivo.
Acid sphingomyelinase possesses a domain homologous to its activator proteins: saposins B and D.
Acid sphingomyelinase, cell membranes and human disease: lessons from Niemann-Pick disease.
Acid sphingomyelinase: identification of nine novel mutations among Italian Niemann Pick type B patients and characterization of in vivo functional in-frame start codon.
Acidic sphingomyelinase (ASM) is necessary for fas-induced GD3 ganglioside accumulation and efficient apoptosis of lymphoid cells.
Activation of human acid sphingomyelinase through modification or deletion of C-terminal cysteine.
Adrenal changes in Niemann-Pick disease: differences between sphingomyelinase deficiency and type C.
Adult sphingomyelinase deficiency: report of 2 patients who initially presented with psychiatric disorders.
Adult-onset pulmonary involvement in Niemann-Pick disease type B.
Alleged Detrimental Mutations in the SMPD1 Gene in Patients with Niemann-Pick Disease.
Allogeneic bone marrow-plus-liver transplantation in the C57BL/KsJ spm/spm mouse, an animal model of Niemann-Pick disease.
Alpha galactosidase A activity in Parkinson's disease.
Alterations of myelin-specific proteins and sphingolipids characterize the brains of acid sphingomyelinase-deficient mice, an animal model of Niemann-Pick disease type A.
Altered Lipid Metabolism in Brain Injury and Disorders.
Altered lipid metabolism in brain injury and disorders.
Alveolar lipoproteinosis in an acid sphingomyelinase-deficient mouse model of Niemann-Pick disease.
An Early-Onset Neuronopathic Form of Acid Sphingomyelinase Deficiency: A SMPD1 p.C133Y Mutation in the Saposin Domain of Acid Sphingomyelinase.
An enzymatic assay for quantifying sphingomyelin in tissues and plasma from humans and mice with Niemann-Pick disease.
An induced pluripotent stem cell line (TRNDi009-C) from a Niemann-Pick disease type A patient carrying a heterozygous p.L302P (c.905 T>C) mutation in the SMPD1 gene.
An ultramicrochemical assay for sphingomyelinase: rapid prenatal diagnosis of a fetus at risk for Niemann-Pick disease.
Analysis of Acid sphingomyelinase activity in dried blood spots using tandem mass spectrometry.
Analysis of the lung pathology and alveolar macrophage function in the acid sphingomyelinase--deficient mouse model of Niemann-Pick disease.
Analysis of the sphingomyelin phosphodiesterase 1 gene (SMPD1) in Turkish Niemann-Pick disease patients: mutation profile and description of a novel mutation.
Anti-human class I MHC antibodies induce apoptosis by a pathway that is distinct from the Fas antigen-mediated pathway.
Assay of beta-glucosidase and sphingomyelinase for identification of patients and carriers of Gaucher's and Niemann-Pick diseases.
Autopsy pathology of infantile neurovisceral ASMD (Niemann-Pick Disease type A): Clinicopathologic correlations of a case report.
Bi-functional IgG-lysosomal enzyme fusion proteins for brain drug delivery.
Biochemical and ultrastructural findings in a lymphoid cell line from Niemann-Pick disease type A.
Biochemical and ultrastructural studies on an Epstein-Barr virus-transformed lymphoid cell line from a Niemann-Pick disease type C patient.
Biochemical studies in Niemann-Pick disease. III. In vitro and in vivo assays of sphingomyelin degradation in cultured skin fibroblasts and amniotic fluid cells for the diagnosis of the various forms of the disease.
Biochemical, pathological, and clinical response to transplantation of normal bone marrow cells into acid sphingomyelinase-deficient mice.
Biosynthesis of sphingomyelinase in normal and Niemann-Pick fibroblasts.
Bone marrow transplantation in acid sphingomyelinase-deficient mice: engraftment and cell migration into the brain as a function of radiation, age, and phenotype.
Brain pathology in Niemann Pick disease type A: insights from the acid sphingomyelinase knockout mice.
Cathepsin B overexpression due to acid sphingomyelinase ablation promotes liver fibrosis in Niemann-Pick disease.
CD95 (Fas/APO-1) induces ceramide formation and apoptosis in the absence of a functional acid sphingomyelinase.
Cell autonomous apoptosis defects in acid sphingomyelinase knockout fibroblasts.
Changes in PCSK 9 and apolipoprotein B100 in Niemann-Pick disease after enzyme replacement therapy with olipudase alfa.
Characterization of common SMPD1 mutations causing types A and B Niemann-Pick disease and generation of mutation-specific mouse models.
Characterization of human acid sphingomyelinase purified from the media of overexpressing Chinese hamster ovary cells.
Characterization of Niemann-Pick diseases genes mutation spectrum in Iran and identification of a novel mutation in SMPD1 gene.
Chemical and biochemical studies in fetuses affected with Nieman-Pick disease type A.
Chemical and biochemical studies in human fetuses affected with Niemann-Pick disease type A.
Chromatofocusing of skin fibroblast sphingomyelinase: alterations in Niemann-Pick disease type C shared by GM1-gangliosidosis.
Chronic Niemann-Pick disease with sphingomyelinase deficiency in two brothers with mental retardation.
Clathrin-mediated endocytosis is impaired in type A-B Niemann-Pick disease model cells and can be restored by ICAM-1-mediated enzyme replacement.
Clearance of Hepatic Sphingomyelin by Olipudase Alfa Is Associated With Improvement in Lipid Profiles in Acid Sphingomyelinase Deficiency.
Clinical and biochemical diagnostics of Niemann-Pick disease.
Clinical relevance of endpoints in clinical trials for acid sphingomyelinase deficiency enzyme replacement therapy.
Clinical, biochemical, and genotype-phenotype correlations of 118 patients with Niemann-Pick disease Types A/B.
Combination brain and systemic injections of AAV provide maximal functional and survival benefits in the Niemann-Pick mouse.
Combination-targeting to multiple endothelial cell adhesion molecules modulates binding, endocytosis, and in vivo biodistribution of drug nanocarriers and their therapeutic cargoes.
Combined Emphysema and Interstitial Lung Disease as a Rare Presentation of Pulmonary Involvement in a Patient with Chronic Visceral Acid Sphingomyelinase Deficiency (Niemann-Pick Disease Type B).
Comparative binding, endocytosis, and biodistribution of antibodies and antibody-coated carriers for targeted delivery of lysosomal enzymes to ICAM-1 versus transferrin receptor.
Comparative effects of recombinant acid sphingomyelinase administration by different routes in niemann-pick disease mice.
Compound heterozygosity at the sphingomyelin phosphodiesterase-1 (SMPD1) gene is associated with low HDL cholesterol.
Comprehensive Evaluation of Plasma 7-Ketocholesterol and Cholestan-3?,5?,6?-Triol in an Italian Cohort of Patients Affected by Niemann-Pick Disease due to NPC1 and SMPD1 Mutations.
Connecting Hsp70, sphingolipid metabolism and lysosomal stability.
Creation of a mouse model for non-neurological (type B) Niemann-Pick disease by stable, low level expression of lysosomal sphingomyelinase in the absence of secretory sphingomyelinase: relationship between brain intra-lysosomal enzyme activity and central nervous system function.
Critical assessment of chitotriosidase analysis in the rational laboratory diagnosis of children with Gaucher disease and Niemann-Pick disease type A/B and C.
Crystal structure of mammalian acid sphingomyelinase.
Cutting Edge of the Pathogenesis of Atopic Dermatitis: Sphingomyelin Deacylase, the Enzyme Involved in Its Ceramide Deficiency, Plays a Pivotal Role.
Decreasing SMPD1 activity in BEAS-2B bronchial airway epithelial cells results in increased NRF2 activity, cytokine synthesis and neutrophil recruitment.
Deep sequencing of SMPD1 gene revealed a heterozygous frameshift mutation (p.Ser192Alafs) in a Palestinian infant with Niemann-Pick disease type A: a case report.
Defective Autophagy, Mitochondrial Clearance and Lipophagy in Niemann-Pick Type B Lymphocytes.
Defects in sarcolemma repair and skeletal muscle function after injury in a mouse model of Niemann-Pick type A/B disease.
Deficiency of taurocholate-dependent phospholipase C acting on phosphatidylcholine in Niemann-Pick disease.
Degradation of lysophosphatidylcholine by lysosomes. Stimulation of lysophospholipase C by taurocholate and deficiency in Niemann-Pick fibroblasts.
Deletion of arginine (608) in acid sphingomyelinase is the prevalent mutation among Niemann-Pick disease type B patients from northern Africa.
Delivery of acid sphingomyelinase in normal and niemann-pick disease mice using intercellular adhesion molecule-1-targeted polymer nanocarriers.
Depletion of Host and Viral Sphingomyelin Impairs Influenza Virus Infection.
Determination of 7-ketocholesterol in plasma by LC-MS for rapid diagnosis of acid SMase-deficient Niemann-Pick disease.
Diagnosis of Niemann-Pick disease using a simple and sensitive fluorimetric assay of sphingomyelinase activity.
Dot immunodetection for sphingomyelinase with monoclonal antibody.
Dual diagnosis of Ochoa syndrome and Niemann-Pick disease type B in a consanguineous family.
Effect of host cell lipid metabolism on alphavirus replication, virion morphogenesis, and infectivity.
Effects of dimethylsulfoxide on sphingomyelinase activities in normal and Niemann-Pick type A, B and C fibroblasts.
Effects of sphingomyelin/ceramide ratio on the permeability and microstructure of model stratum corneum lipid membranes.
Elevated sphingomyelinase and hypercytokinemia in hemophagocytic lymphohistiocytosis.
Enhanced Delivery and Effects of Acid Sphingomyelinase by ICAM-1-Targeted Nanocarriers in Type B Niemann-Pick Disease Mice.
Enzyme activities and phospholipid storage patterns in brain and spleen samples from Niemann-Pick disease variants: a comparison of neuropathic and non-neuropathic forms.
Epidemiological, clinical and biochemical characterization of the p.(Ala359Asp) SMPD1 variant causing Niemann-Pick disease type B.
ESI-MS quantitation of increased sphingomyelin in Niemann-Pick disease type B HDL.
Evidence for the association of ultraviolet-C and H(2)O(2)-induced apoptosis with acid sphingomyelinase activation.
Excess sphingomyelin disturbs ATG9A trafficking and autophagosome closure.
Facile Method for Specifically Sensing Sphingomyelinase in Cells and Human Urine Based on a Ratiometric Fluorescent Nanoliposome Probe.
Fatal Postpartum Hemorrhage in a Patient with Niemann-Pick Disease Type B.
Feline Niemann-Pick Disease With a Novel Mutation of SMPD1 Gene.
Fluorescence-based selection of retrovirally transduced cells in the absence of a marker gene: direct selection of transduced type B Niemann-Pick disease cells and evidence for bystander correction.
Four novel p.N385K, p.V36A, c.1033-1034insT and c.1417-1418delCT mutations in the sphingomyelin Phosphodiesterase 1 (SMPD1) gene in patients with types A and B Niemann-Pick disease (NPD).
Functional analysis of acid and neutral sphingomyelinases in vitro and in vivo.
Functional characterization of novel variants in SMPD1 in Indian patients with acid sphingomyelinase deficiency.
Functional in vitro characterization of 14 SMPD1 mutations identified in Italian patients affected by Niemann Pick Type B disease.
Gelastic cataplexy in Niemann-Pick disease group C and related variants without generalized sphingomyelinase deficiency.
Generation of an induced pluripotent stem cell line (TRNDi004-I) from a Niemann-Pick disease type B patient carrying a heterozygous mutation of p.L43_A44delLA in the SMPD1 gene.
Hematopoietic stem cell gene therapy leads to marked visceral organ improvements and a delayed onset of neurological abnormalities in the acid sphingomyelinase deficient mouse model of Niemann-Pick disease.
Hepatosplenomegaly, pneumopathy, bone changes and fronto-temporal dementia: Niemann-Pick type B and SQSTM1-associated Paget's disease in the same individual.
High performance liquid chromatography for the detection of homozygotes and heterozygotes of Niemann-Pick disease.
High sphingomyelin levels induce lysosomal damage and autophagy dysfunction in Niemann Pick disease type A.
Highly variable neural involvement in sphingomyelinase-deficient Niemann-Pick disease caused by an ancestral Gypsy mutation.
Hsp70 stabilizes lysosomes and reverts Niemann-Pick disease-associated lysosomal pathology.
Human acid sphingomyelinase structures provide insight to molecular basis of Niemann-Pick disease.
Human acid sphingomyelinase.
ICAM-1 targeting, intracellular trafficking, and functional activity of polymer nanocarriers coated with a fibrinogen-derived peptide for lysosomal enzyme replacement.
Identification and characterization of eight novel SMPD1 mutations causing types A and B Niemann-Pick disease.
Identification and characterization of SMPD1 mutations causing Niemann-Pick types A and B in Spanish patients.
Identification and expression of a common missense mutation (L302P) in the acid sphingomyelinase gene of Ashkenazi Jewish type A Niemann-Pick disease patients.
Identification and expression of a missense mutation (Y446C) in the acid sphingomyelinase gene from a Japanese patient with type A Niemann-Pick disease.
Identification and expression of five mutations in the human acid sphingomyelinase gene causing types A and B Niemann-Pick disease. Molecular evidence for genetic heterogeneity in the neuronopathic and non-neuronopathic forms.
Identification of a 3' acceptor splice site mutation (g2610c) in the acid sphingomyelinase gene of patients with Niemann-Pick disease.
Identification of a distinct mutation spectrum in the SMPD1 gene of Chinese patients with acid sphingomyelinase-deficient Niemann-Pick disease.
Identification of a missense mutation (S436R) in the acid sphingomyelinase gene from a Japanese patient with type B Niemann-Pick disease.
Identification of novel biomarkers for niemann-pick disease using gene expression analysis of Acid sphingomyelinase knockout mice.
Identification of seven novel SMPD1 mutations causing Niemann-Pick disease types A and B.
Imaging of pulmonary manifestations in subtype B of Niemann-Pick disease.
Immunological studies on lysosomal sphingomyelinase: identification of a 28 000-Da component deficient in urine from patients with Niemann-Pick disease types A and B.
Imprinting at the SMPD1 locus: implications for acid sphingomyelinase-deficient Niemann-Pick disease.
Improved sensitivity of an acid sphingomyelinase activity assay using a C6:0 sphingomyelin substrate.
Improvement of biochemical and behavioral defects in the Niemann-Pick type A mouse by intraventricular infusion of MARCKS.
In Silico Analysis of the Molecular-Level Impact of SMPD1 Variants on Niemann-Pick Disease Severity.
Induced Pluripotent Stem Cells for Disease Modeling and Evaluation of Therapeutics for Niemann-Pick Disease Type A.
Infusion of recombinant human acid sphingomyelinase into niemann-pick disease mice leads to visceral, but not neurological, correction of the pathophysiology.
Interstitial lung disease in lysosomal storage disorders.
Intertwined mechanisms define transport of anti-ICAM nanocarriers across the endothelium and brain delivery of a therapeutic enzyme.
Intracerebral transplantation of adult mouse neural progenitor cells into the Niemann-Pick-A mouse leads to a marked decrease in lysosomal storage pathology.
Intracerebral transplantation of mesenchymal stem cells into acid sphingomyelinase-deficient mice delays the onset of neurological abnormalities and extends their life span.
In vivo performance of polymer nanocarriers dually-targeted to epitopes of the same or different receptors.
Isoenzymes of sphingomyelinase and the genetic defect in Niemann-Pick disease, type C.
Juvenile dystonic lipidosis (variant of Niemann-Pick disease type C).
Lack of Acid Sphingomyelinase Induces Age-Related Retinal Degeneration.
Letters: A simple sphingomyelinase determination for Niemann-Pick disease: differential diagnosis of types A, B and C.
Light and electron microscopic analysis of the central and peripheral nervous systems of acid sphingomyelinase-deficient mice resulting from gene targeting.
Limited benefits of presymptomatic cord blood transplantation in neurovisceral acid sphingomyelinase deficiency (ASMD) intermediate type.
Liposome-targeted recombinant human acid sphingomyelinase: Production, formulation, and in vitro evaluation.
Liver and skin histopathology in adults with acid sphingomyelinase deficiency (Niemann-Pick disease type B).
Lyso-sphingomyelin is elevated in dried blood spots of Niemann-Pick B patients.
Lysosomal enzyme delivery by ICAM-1-targeted nanocarriers bypassing glycosylation- and clathrin-dependent endocytosis.
Lysosomal involvement in cellular turnover of plasma membrane sphingomyelin.
Macula halo syndrome and non-pitting lid oedema in an Italian family.
Macula halo syndrome.
Macula halo syndrome. Variant of Niemann-Pick disease.
Mannose 6-phosphate receptor-mediated uptake is defective in acid sphingomyelinase-deficient macrophages: implications for Niemann-Pick disease enzyme replacement therapy.
Merits of Combination Cortical, Subcortical, and Cerebellar Injections for the Treatment of Niemann-Pick Disease Type A.
Modulating cancer cell survival by targeting intracellular cholesterol transport.
Modulation of subcellular distribution of doxorubicin in multidrug-resistant P388/ADR mouse leukemia cells by the chemosensitizer ((2-isopropyl-1-(4-[3-N-methyl-N-(3,4-dimethoxy-beta- phenethyl)amino]propyloxy)-benzenesulfonyl))indolizine.
Molecular analysis of the acid sphingomyelinase deficiency in a family with an intermediate form of Niemann-Pick disease.
Molecular basis of acid sphingomyelinase deficiency in a patient with Niemann-Pick disease type A.
Molecular forms of sphingomyelinase and non-specific phosphodiesterases in Epstein-Barr virus-transformed lymphoid cell lines from Niemann-Pick disease types A and B.
Molecular genetic characterization of novel sphingomyelin phosphodiesterase 1 mutations causing niemann-pick disease.
Morphological diagnosis of Niemann-Pick disease type C by skin and conjunctival biopsies.
MRI-guided delivery of AAV2 to the primate brain for the treatment of lysosomal storage disorders.
Mutational spectrum of SMPD1 gene in Pakistani Niemann-Pick disease patients.
Neurofibrillary tangles in Niemann-Pick disease type C.
New Single Nucleotide Deletion In the SMPD1 Gene Causes Niemann Pick Disease Type A in a Child from Southwest Iran: A Case Report.
New tools for the study of Niemann-Pick disease: analogues of natural substrate and Epstein-Barr virus-transformed lymphoid cell lines.
Niemann Pick Disease type A in Israeli Arabs: 677delT, a common novel single mutation. Mutations in brief no. 161. Online.
Niemann Pick disease: presence of the magnesium-dependent sphingomyelinase in brain of the infantile form of the disease.
Niemann-Pick disease (variation in the sphingomyelinase deficient group). Neurovisceral phenotype (A) with an abnormally protracted clinical course and variable expression of neurological symptomatology in three siblings.
Niemann-Pick disease A or B in four pediatric patients and SMPD1 mutation carrier frequency in the Mexican population.
Niemann-Pick disease and juvenile xanthogranuloma. Are they related?
Niemann-Pick disease experimental model: sphingomyelinase reduction induced by AY-9944.
Niemann-Pick disease in a poodle dog.
Niemann-Pick disease type A and B are clinically but also enzymatically heterogeneous: pitfall in the laboratory diagnosis of sphingomyelinase deficiency associated with the mutation Q292 K.
Niemann-Pick disease type B: an unusual clinical presentation with multiple vertebral fractures.
Niemann-Pick disease type B: first-trimester prenatal diagnosis on chorionic villi and biochemical study of a foetus at 12 weeks of development.
Niemann-Pick disease type B: prenatal diagnosis and enzymatic and chemical studies on fetal brain and liver.
Niemann-Pick disease type C presenting as neonatal hepatitis: report of one case.
Niemann-Pick disease type C: an update.
Niemann-Pick disease type D: lipid analyses and studies on sphingomyelinases.
Niemann-Pick disease type-B: a unique case report with compound heterozygosity and complicated lipid management.
Niemann-Pick Disease versus acid sphingomyelinase deficiency.
Niemann-Pick disease, type C and Roscoe Brady.
Niemann-Pick disease, Type C: evidence for the deficiency of an activating factor stimulating sphingomyelin and glucocerebroside degradation.
Niemann-Pick disease.
Niemann-Pick disease: a frequent missense mutation in the acid sphingomyelinase gene of Ashkenazi Jewish type A and B patients.
Niemann-Pick disease: coupling and uncoupling of inhibited sphingomyelinase activity and exogenous cholesterol esterification in fibroblasts by ionophore treatment.
Niemann-Pick disease: mutation update, genotype/phenotype correlations, and prospects for genetic testing.
Niemann-Pick disease: prenatal diagnoses and studies of sphingomyelinase activities.
Niemann-Pick diseases.
Niemann-Pick human lymphoblasts are resistant to phthalocyanine 4-photodynamic therapy-induced apoptosis.
Niemann-Pick type B disease. Identification of a single codon deletion in the acid sphingomyelinase gene and genotype/phenotype correlations in type A and B patients.
Niemann-Pick's disease. Clinical, biochemical and ultrastructural findings in a case of the infantile form.
Niemann–Pick disease: own observations and new therapeutic options
Novel first-dose adverse drug reactions during a phase I trial of olipudase alfa (recombinant human acid sphingomyelinase) in adults with Niemann-Pick disease type B (acid sphingomyelinase deficiency).
Novel mutations in the SMPD1 gene in Jordanian children with Acid sphingomyelinase deficiency (Niemann-Pick types A and B).
Ophthalmologic manifestations of type B Niemann-Pick diseases.
Optimization of a histopathological biomarker for sphingomyelin accumulation in Acid sphingomyelinase deficiency.
Orthotopic liver transplantation in two adults with Niemann-Pick and Gaucher's diseases: implications for the treatment of inherited metabolic disease.
Pathogenic Compound Heterozygous Mutations in a Mexican Mestizo Patient with Niemann-Pick Disease Type B.
Pathology of visceral organs and bone marrow in an acid sphingomyelinase deficient knock-out mouse line, mimicking human Niemann-Pick disease type A. A light and electron microscopic study.
Peripheral neuropathy in type A Niemann-Pick disease. A morphological study.
Pharmacological reversion of sphingomyelin-induced dendritic spine anomalies in a Niemann Pick disease type A mouse model.
Phosphatidylinositol-3,5-Bisphosphate is a potent and selective inhibitor of acid sphingomyelinase.
Preimplantation diagnosis of a lysosomal storage disorder by in situ enzymatic activity: 'proof of principle' in acid sphingomyelinase-deficient mice.
Preimplantation genetic diagnosis for Niemann-Pick disease type B.
Prenatal diagnosis and fetal pathology of Niemann-Pick disease.
Prenatal diagnosis of Gaucher's and Niemann-Pick diseases. Assays of glucocerebrosidase and sphingomyelinase in tissue cultures using natural substrates.
Prenatal diagnosis of Niemann-Pick disease type C.
Prenatal diagnosis of Niemann-Pick diseases types A, B and C.
Presence of immunoreactive material in Niemann-Pick type A placenta using anti-sphingomyelinase rabbit gammaglobulins.
Prospective study of the natural history of chronic acid sphingomyelinase deficiency in children and adults: eleven years of observation.
Pulmonary delivery of recombinant acid sphingomyelinase improves clearance of lysosomal sphingomyelin from the lungs of a murine model of Niemann-Pick disease.
Purification and characterization of recombinant, human acid ceramidase. Catalytic reactions and interactions with acid sphingomyelinase.
Quantitation of plasmatic lysosphingomyelin and lysosphingomyelin-509 for differential screening of Niemann-Pick A/B and C diseases.
R542X mutation in SMPD1 gene: genetically novel mutation with phenotypic features intermediate between type A and type B Niemann-Pick disease.
Rapid screening for lipid storage disorders using biochemical markers. Expert center data and review of the literature.
Recombinant human acid sphingomyelinase as an adjuvant to sorafenib treatment of experimental liver cancer.
Reduced cellular cholesterol efflux and low plasma high-density lipoprotein cholesterol in a patient with type B Niemann-Pick disease because of a novel SMPD-1 mutation.
Regional assignment of the human acid sphingomyelinase gene (SMPD1) by PCR analysis of somatic cell hybrids and in situ hybridization to 11p15.1----p15.4.
Regulation of the NPC2 protein-mediated cholesterol trafficking by membrane lipids.
Replacement therapy for inherited enzyme deficiency: liver orthotopic transplantation in Niemann-Pick disease type A.
Reproductive pathology and sperm physiology in acid sphingomyelinase-deficient mice.
Resolution of tissue sphingomyelinase isoelectric profile in multiple components is extraction-dependent: evidence for a component defect in Niemann-Pick disease type C is spurious.
Respiratory impairment in Niemann-Pick B disease: Two case reports and review for the pulmonologist.
Retroviral-mediated transfer of the human acid sphingomyelinase cDNA: correction of the metabolic defect in cultured Niemann-Pick disease cells.
Role of sphingomyelinases in neurological disorders.
Roles and regulation of secretory and lysosomal acid sphingomyelinase.
Roles and Regulation of Secretory and Lysosomal Acid Sphingomyelinase.
Safety study of adeno-associated virus serotype 2-mediated human Acid sphingomyelinase expression in the nonhuman primate brain.
Screening of 25 Italian patients with Niemann-Pick A reveals fourteen new mutations, one common and thirteen private, in SMPD1.
Secretory sphingomyelinase in health and disease.
Serum Zn(2+)-stimulated sphingomyelinase deficiency in type B Niemann-Pick disease.
Seven novel acid sphingomyelinase gene mutations in Niemann-Pick type A and B patients.
Seven novel mutations of the SMPD1 gene in four Chinese patients with Niemann-Pick disease type A and prenatal diagnosis for four fetuses.
Signaling pathway for radiation-induced apoptosis in the lymphoblasts from neuronopathic (type A) and non-neuronopathic (type B) forms of Niemann-Pick disease.
Skeletal manifestations in pediatric and adult patients with Niemann Pick disease type B.
SMPD1 variants in Chinese Han patients with sporadic Parkinson's disease.
Solving the secretory acid sphingomyelinase puzzle: Insights from lysosome-mediated parasite invasion and plasma membrane repair.
Somatic cell hybridisation studies showing different gene mutations in Niemann-Pick variants.
Spectrum of SMPD1 mutations in Asian-Indian patients with acid sphingomyelinase (ASM)-deficient Niemann-Pick disease.
Sperm Abnormalities in Heterozygous Acid Sphingomyelinase Knockout Mice Reveal a Novel Approach for the Prevention of Genetic Diseases.
Sperm abnormalities in heterozygous acid sphingomyelinase knockout mice reveal a novel approach for the prevention of genetic diseases.
Sphingomyelin phosphodiesterase-1 (SMPD1) coding variants do not contribute to low levels of high-density lipoprotein cholesterol.
Sphingomyelin storage in a patient with myoclonus epilepsy as a main clinical symptom -- a varient in Niemann-Pick disease type C.
Sphingomyelinase activities in cultured skin fibroblasts from patients with Niemann-Pick Disease.
Sphingomyelinase activities of various human tissues in control subjects and in Niemann-Pick disease - development and evaluation of a microprocedure.
Sphingomyelinase activity levels in human peripheral blood leukocytes, using [3H]sphingomyelin as substrate: study of heterozygotes and homozygotes for Niemann-Pick disease variants.
Sphingomyelinase and nonspecific phosphodiesterase activities in Epstein-Barr virus-transformed lymphoid cell lines from Niemann-Pick disease A, B and C.
Sphingomyelinase defect in Niemann-Pick disease, type C, fibroblasts.
Sphingomyelinase enzyme assay in Niemann-Pick disease.
Sphingomyelinase in cultured skin fibroblasts from normal and Niemann-Pick type C patients.
Sphingomyelinase in normal human spleens and in spleens from subjects with Niemann-Pick disease.
Sphingomyelinase isozymes of human tissues: a phpothesis on enzymatic differentiation of the neuropathic and non-neuropathic forms of Niemann-Pick disease.
Structural and functional analysis of the ASM p.Ala359Asp mutant that causes acid sphingomyelinase deficiency.
Studies on pyrophosphate diesterase activity in cultured human fibroblasts: a deficiency in Niemann-Pick disease.
Studies on sphingomyelinase activity in cultured cells and leucocytes.
Studies on sphingomyelinase and beta-glucosidase activities in Niemann-Pick disease variants. Phosphodiesterase activities measured with natural and artificial substrates.
Subclinical course of adult visceral Niemann-Pick type C1 disease. A rare or underdiagnosed disorder?
Successful within-patient dose escalation of olipudase alfa in acid sphingomyelinase deficiency.
Synaptic vesicle docking: sphingosine regulates syntaxin1 interaction with Munc18.
Synthesis of pyrenesulfonylamido-sphingomyelin and its use as substrate for determining sphingomyelinase activity and diagnosing Niemann-Pick disease.
Systemic lupus erythematosus occurring in a patient with Niemann-Pick type B disease.
The Acid Sphingomyelinase Sequence Variant p.A487V Is Not Associated With Decreased Levels of Enzymatic Activity.
The IL-1 receptor signaling pathway.
The impact of biomarkers analysis in the diagnosis of Niemann-Pick C disease and acid sphingomyelinase deficiency.
The natural history of type B Niemann-Pick disease: results from a 10-year longitudinal study.
The pathogenesis and treatment of acid sphingomyelinase-deficient Niemann-Pick disease.
The R608del mutation in the acid sphingomyelinase gene (SMPD1) is the most prevalent among patients from Gran Canaria Island with Niemann-Pick disease type B.
The structure and catalytic mechanism of human sphingomyelin phosphodiesterase like 3a--an acid sphingomyelinase homologue with a novel nucleotide hydrolase activity.
The unexpected role of acid sphingomyelinase in cell death and the pathophysiology of common diseases.
Toward gene therapy for Niemann-Pick disease (NPD): separation of retrovirally corrected and noncorrected NPD fibroblasts using a novel fluorescent sphingomyelin.
Transitory type of sphingomyelinase deficient Niemann-Pick disease: clinical and morphological studies and follow-up of two sisters.
Treatment of sphingomyelinase deficiency by repeated implantations of amniotic epithelial cells.
Two new mutations in the acid sphingomyelinase gene causing type a Niemann-pick disease: N389T and R441X.
Two Siblings with Niemann-Pick Disease (NPD) Type B: Clinical Findings and Novel Mutations of the Acid Sphingomyelinase Gene.
Type A Niemann-Pick disease: a frameshift mutation in the acid sphingomyelinase gene (fsP330) occurs in Ashkenazi Jewish patients.
Unsuccessful treatment attempt: cord blood stem cell transplantation in a patient with Niemann-Pick disease type A.
Uptake and intracellular degradation of fluorescent sphingomyelin by fibroblasts from normal individuals and a patient with Niemann-Pick disease.
Uptake and metabolism of radioactively labeled sphingomyelin in cultured skin fibroblasts from controls and patients with Niemann-Pick disease and other lysosomal storage diseases.
Zn2+-stimulated sphingomyelinase is secreted by many cell types and is a product of the acid sphingomyelinase gene.
[A Case of a Korean Adult Affected by Type B Niemann-Pick Disease: Secondary Sea-blue Histiocytosis and Molecular Characterization.]
[A case of type C Niemann-Pick disease]
[A female patient with splenomegaly, interstitial pneumopathy and giant foam cells in bone marrow]
[A novel mutation in two spanish children with the Niemann Pick disease: Description of genotype, acid sphingomyelinase activity, phenotype and review].
[Activators for sphingohydrolases and the nature of the sphingomyelinase deficiency in Niemann-Pick disease types A, B and C (author's transl)]
[Adult Niemann-Pick disease: a 26 years follow-up. Report of a case with isolated visceral involvement, excess of tissue sphingomyelin, and deficient sphingomyelinase activity (author's transl)]
[Bone marrow manifestation of Niemann-Pick disease. A review of histiocytic proliferations of the bone marrow]
[Interstitial pneumopathy revealing type B Niemann Pick disease in an adult]
[Niemann-Pick disease revealed late by miliary tuberculosis: diagnosis by leukocyte determination of sphingomyelinase deficiency]
[Niemann-Pick disease type B identified following an episode of bronchopneumonia]
[Niemann-Pick disease type B: An enzymatically confirmed case with unexpected retinal involvement (author's transl)]
[Niemann-Pick disease type C (subacute neurovisceral lipidosis). Problems of altered sphingomyelinase activity in the brain (author's transl)]
[Niemann-Pick disease types A and B]
[Niemann-Pick disease [type A and B] (acid sphingomyelinase deficiencies)]
[Sphingomyelinase activity in a patient with Niemann-Pick disease]
[Type B Niemann Pick disease: clinical description of three patients in a same family]
Non-alcoholic Fatty Liver Disease
Amitriptyline inhibits nonalcoholic steatohepatitis and atherosclerosis induced by high-fat diet and LPS through modulation of sphingolipid metabolism.
High fat diet induced hepatic steatosis and insulin resistance: Role of dysregulated ceramide metabolism.
Macrophage-derived thrombospondin 1 promotes obesity-associated non-alcoholic fatty liver disease.
Serum acid sphingomyelinase is upregulated in chronic hepatitis C infection and non alcoholic fatty liver disease.
Sphingomyelinases and Liver Diseases.
Obesity
A high-throughput sphingomyelinase assay using natural substrate.
Altered adipose and plasma sphingolipid metabolism in obesity: a potential mechanism for cardiovascular and metabolic risk.
Genome Regulation and Gene Interaction Networks Inferred From Muscle Transcriptome Underlying Feed Efficiency in Pigs.
Genome-Wide DNA Methylation Profiles of Phlegm-Dampness Constitution.
Pioglitazone time-dependently reduces tumour necrosis factor-alpha level in muscle and improves metabolic abnormalities in Wistar fatty rats.
Serum sphingolipids and inflammatory mediators in adolescents at risk for metabolic syndrome.
Sortilin deficiency improves the metabolic phenotype and reduces hepatic steatosis of mice subjected to diet-induced obesity.
Sphingomyelinases and Liver Diseases.
Ocular Hypertension
Evidence for ceramide induced cytotoxicity in retinal ganglion cells.
Oligodendroglioma
Acid sphingomyelinase and inhibition by phosphate ion: role of inhibition by phosphatidyl-myo-inositol 3,4,5-triphosphate in oligodendrocyte cell signaling.
Differential regulation of sphingomyelin synthesis and catabolism in oligodendrocytes and neurons.
Regulation of sphingomyelinases in cells of the oligodendrocyte lineage.
The hyaluronic acid inhibitor 4-methylumbelliferone is an NSMase2 activator-role of Ceramide in MU anti-tumor activity.
Osteogenesis Imperfecta
Hyaluronan synthase-2 upregulation protects smpd3-deficient fibroblasts against cell death induced by nutrient deprivation, but not against apoptosis evoked by oxidized LDL.
Inducible transient expression of Smpd3 prevents early lethality in fro/fro mice.
Local regulation of tooth mineralization by sphingomyelin phosphodiesterase 3.
Neutral Sphingomyelinase 2 (SMPD3) Deficiency in Mice Causes Chondrodysplasia with Unimpaired Skeletal Mineralization.
Neutral sphingomyelinase 2 deficiency is associated with lung anomalies similar to emphysema.
Smpd3 Expression in both Chondrocytes and Osteoblasts Is Required for Normal Endochondral Bone Development.
Osteomyelitis
Capsule expression and genotypic differences among Staphylococcus aureus isolates from patients with chronic or acute osteomyelitis.
The role of antibodies against alpha-toxin and teichoic acid in the diagnosis of staphylococcal infections.
Osteoporosis
Neutral Sphingomyelinase 2 (SMPD3) Deficiency in Mice Causes Chondrodysplasia with Unimpaired Skeletal Mineralization.
Osteosarcoma
Lactosylceramide is required in apoptosis induced by N-Smase.
Mutations in the neutral sphingomyelinase gene SMPD3 implicate the ceramide pathway in human leukemias.
Ototoxicity
Impact of sphingolipid mediators on determination of cochlear survival in ototoxicity.
Ovarian Neoplasms
A novel cisplatin mediated apoptosis pathway is associated with acid sphingomyelinase and FAS proapoptotic protein activation in ovarian cancer.
Acid sphingomyelinase, a novel negative biomarker of ovarian cancer.
Pancreatic Neoplasms
?-Tocotrienol induces apoptosis in pancreatic cancer cells by upregulation of ceramide synthesis and modulation of sphingolipid transport.
Pancreatitis
Effect of staphylococcal alpha-toxin pancreatitis on glucose tolerance in the dog.
Paralysis
[Lipidosis with vertical gaze palsy, macular degeneration, and sphingomyelinase deficiency]
Parasitemia
Influence of amitriptyline on eryptosis, parasitemia and survival of Plasmodium berghei-infected mice.
T Cell-Specific Overexpression of Acid Sphingomyelinase Results in Elevated T Cell Activation and Reduced Parasitemia During Plasmodium yoelii Infection.
Parkinson Disease
A rare lysosomal enzyme gene SMPD1 variant (p.R591C) associates with Parkinson's disease.
Association study between SMPD1 p.L302P and sporadic Parkinson's disease in ethnic Chinese population.
Chromosomal microarray and whole-exome sequence analysis in Taiwanese patients with autism spectrum disorder.
Correction to: Pharmacological inhibition of nSMase2 reduces brain exosome release and ?-synuclein pathology in a Parkinson's disease model.
Excessive burden of lysosomal storage disorder gene variants in Parkinson's disease.
Genetic convergence of Parkinson's disease and lysosomal storage disorders.
Genetic risk factors in Finnish patients with Parkinson's disease.
Interactions between neural membrane glycerophospholipid and sphingolipid mediators: a recipe for neural cell survival or suicide.
LRRK2, GBA and SMPD1 Founder Mutations and Parkinson's Disease in Ashkenazi Jews.
Neutral Sphingomyelinase Behaviour in Hippocampus Neuroinflammation of MPTP-Induced Mouse Model of Parkinson's Disease and in Embryonic Hippocampal Cells.
Pharmacological inhibition of nSMase2 reduces brain exosome release and ?-synuclein pathology in a Parkinson's disease model.
SMPD1 mutations and Parkinson disease.
SMPD1 mutations, activity, and ?-synuclein accumulation in Parkinson's disease.
SMPD1 variants do not have a major role in rapid eye movement sleep behavior disorder.
SMPD1 variants in Chinese Han patients with sporadic Parkinson's disease.
Systematic Genetic Analysis of the SMPD1 Gene in Chinese Patients with Parkinson's Disease.
The contribution of Niemann-Pick SMPD1 mutations to Parkinson disease in Ashkenazi Jews.
The emerging role of SMPD1 mutations in Parkinson's disease: Implications for future studies.
The p.L302P mutation in the lysosomal enzyme gene SMPD1 is a risk factor for Parkinson disease.
[Mutation del 1,02kb in the CLN3 gene and extrapyramidal syndrome].
Periodontitis
Acid sphingomyelinase deficiency exacerbates LPS-induced experimental periodontitis.
Peritonitis
Role of antibodies against fibronectin-, collagen-binding proteins and alphatoxin in experimental Staphylococcus aureus peritonitis and septicaemia in neutropenic mice.
The urine from patients with peritonitis as a rich source for purifying human acid sphingomyelinase and other lysosomal enzymes.
Pheochromocytoma
Alpha-toxin permeabilized rat pheochromocytoma cells: a new approach to investigate stimulus-secretion coupling.
Antioxidant and pro-oxidant capacity of catecholamines and related compounds. Effects of hydrogen peroxide on glutathione and sphingomyelinase activity in pheochromocytoma PC12 cells: potential relevance to age-related diseases.
CrmA protects against apoptosis and ceramide formation in PC12 cells.
Further characterization of dopamine release by permeabilized PC12 cells.
Melatonin Acts as an Antidepressant by Inhibition of the Acid Sphingomyelinase/Ceramide System.
Minimal requirements for exocytosis. A study using PC 12 cells permeabilized with staphylococcal alpha-toxin.
Staphylococcus aureus alpha-toxin activates phospholipases and induces a Ca2+ influx in PC12 cells.
Photosensitivity Disorders
Fumonisin B1 does not prevent apoptosis in A431 human epidermoid carcinoma cells after photosensitization with a silicon phthalocyanine.
Pick Disease of the Brain
Astrocytic ceramide as possible indicator of neuroinflammation.
Pneumonia
Acid sphingomyelinase deficiency attenuates bleomycin-induced lung inflammation and fibrosis in mice.
Acid Sphingomyelinase Inhibition in Stored Erythrocytes Reduces Transfusion-Associated Lung Inflammation.
Alpha-toxin damages the air-blood barrier of the lung in a rat model of Staphylococcus aureus-induced pneumonia.
Alpha-toxin facilitates the generation of CXC chemokine gradients and stimulates neutrophil homing in Staphylococcus aureus pneumonia.
Altered Macrophage Function Associated with Crystalline Lung Inflammation in Acid Sphingomyelinase Deficiency.
Ceramide in Pseudomonas aeruginosa infections and cystic fibrosis.
Ceramide-1-phosphate inhibits cigarette smoke-induced airway inflammation.
Decreased plasma phospholipid concentrations and increased acid sphingomyelinase activity are accurate biomarkers for community-acquired pneumonia.
Group B streptococcal beta-hemolysin promotes injury of lung microvascular endothelial cells.
Linezolid Attenuates Lethal Lung Damage during Postinfluenza Methicillin-Resistant Staphylococcus aureus Pneumonia.
MEDI4893* Promotes Survival and Extends the Antibiotic Treatment Window in a Staphylococcus aureus Immunocompromised Pneumonia Model.
Staphylococcus aureus alpha-toxin mediates general and cell type-specific changes in metabolite concentrations of immortalized human airway epithelial cells.
[Detection of Staphylococcus aureus and Staphylococcus epidermis species specific antigens and antibodies to alpha-toxin in the blood of patients with pneumonia]
Pneumonia, Necrotizing
Effects of Tedizolid Phosphate on Survival Outcomes and Suppression of Production of Staphylococcal Toxins in a Rabbit Model of Methicillin-Resistant Staphylococcus aureus Necrotizing Pneumonia.
Pneumonia, Staphylococcal
Alpha-toxin facilitates the generation of CXC chemokine gradients and stimulates neutrophil homing in Staphylococcus aureus pneumonia.
Prader-Willi Syndrome
Malignant fibrous histiocytoma-pleomorphic sarcoma, NOS gene expression, histology, and clinical course. A pilot study.
Pre-Eclampsia
Ceramide-Induced Lysosomal Biogenesis and Exocytosis in Early-Onset Preeclampsia Promotes Exosomal Release of SMPD1 Causing Endothelial Dysfunction.
Disruption of sphingolipid metabolism augments ceramide-induced autophagy in preeclampsia.
Epitranscriptomic profiling in human placenta: N6-methyladenosine modification at the 5'-untranslated region is related to fetal growth and preeclampsia.
Increased secretory sphingomyelinase activity in the first trimester of pregnancy in women later developing preeclampsia: a nested case-control study.
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Why high cholesterol levels help hematological malignancies: role of nuclear lipid microdomains.
Pregnancy, High-Risk
Prenatal diagnosis and fetal pathology of Niemann-Pick disease.
Prostatic Neoplasms
Caveolin-1 regulates the ASMase/ceramide-mediated radiation response of endothelial cells in the context of tumor-stroma interactions.
Phosphodiesterase SMPDL3B Gene Expression as Independent Outcome Prediction Marker in Localized Prostate Cancer.
Proteinuria
Barley-ß-glucans reduce systemic inflammation, renal injury and aortic calcification through ADAM17 and neutral-sphingomyelinase2 inhibition.
Podocyte pathology and nephropathy - sphingolipids in glomerular diseases.
Rituximab protects podocytes and exerts anti-proteinuric effects in rat adriamycin-induced nephropathy independent of B-lymphocytes.
Rituximab Treatment Prevents the Early Development of Proteinuria following Pig-to-Baboon Xeno-Kidney Transplantation.
Sphingomyelinase-Like Phosphodiesterase 3b Expression Levels Determine Podocyte Injury Phenotypes in Glomerular Disease.
Pseudomonas Infections
Defective Acid Sphingomyelinase Pathway with Pseudomonas aeruginosa Infection in Cystic Fibrosis.
Psoriasis
Staphylococcal toxins and protein A differentially induce cytotoxicity and release of tumor necrosis factor-alpha from human keratinocytes.
The Spectrum of Mild to Severe Psoriasis Vulgaris Is Defined by a Common Activation of IL-17 Pathway Genes, but with Key Differences in Immune Regulatory Genes.
Puberty, Delayed
Neutral sphingomyelinase 2 (smpd3) in the control of postnatal growth and development.
Pulmonary Disease, Chronic Obstructive
Bacterial Sphingomyelinase is a State-Dependent Inhibitor of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR).
Decreased plasma phospholipid concentrations and increased acid sphingomyelinase activity are accurate biomarkers for community-acquired pneumonia.
Epithelial IL-33 appropriates exosome trafficking for secretion in chronic airway disease.
Neutral sphingomyelinase-2, acid sphingomyelinase, and ceramide levels in COPD patients compared to controls.
Structural and functional characterization of endothelial microparticles released by cigarette smoke.
Pulmonary Edema
Group B streptococcal beta-hemolysin promotes injury of lung microvascular endothelial cells.
PAF-mediated pulmonary edema: a new role for acid sphingomyelinase and ceramide.
Pulmonary Fibrosis
Acid sphingomyelinase deficiency attenuates bleomycin-induced lung inflammation and fibrosis in mice.
Ceramide mediates lung fibrosis in cystic fibrosis.
[Acid sphingomyelinase deficiency and spleen trauma: Splenectomy or not splenectomy?]
Purpura, Thrombocytopenic, Idiopathic
Splenic histiocytosis in idiopathic thrombocytopenic purpura: a relative sphingomyelinase deficiency?
Rectal Neoplasms
Reduction in alkaline sphingomyelinase in colorectal tumorigenesis is not related to the APC gene mutation.
REM Sleep Behavior Disorder
SMPD1 variants do not have a major role in rapid eye movement sleep behavior disorder.
Renal Insufficiency
Partial sphingomyelinase deficiency with sea-blue histiocytosis and neurovisceral dysfunction.
[Functional, histological, biochemical renal modifications. Comparative study of dibekacin, gentamicin, tobramycin, netilmicin and amikacin]
Reperfusion Injury
Critical role of acidic sphingomyelinase in murine hepatic ischemia-reperfusion injury.
Homozygous Smpd1 deficiency aggravates brain ischemia/ reperfusion injury by mechanisms involving polymorphonuclear neutrophils, whereas heterozygous Smpd1 deficiency protects against mild focal cerebral ischemia.
Inhibition of neutral sphingomyelinase decreases arachidonic acid mediated inflammation in liver ischemia-reperfusion injury.
Inhibition of neutral sphingomyelinase decreases elevated levels of nitrative and oxidative stress markers in liver ischemia-reperfusion injury.
Neutral sphingomyelinase inhibition alleviates apoptosis, but not ER stress, in liver ischemia-reperfusion injury.
Raft ceramide in molecular medicine.
Sphingomyelinase and ceramide analogs induce vasoconstriction and leukocyte-endothelial interactions in cerebral venules in the intact rat brain: Insight into mechanisms and possible relation to brain injury and stroke.
Sphingomyelinases: their regulation and roles in cardiovascular pathophysiology.
The Roles of Neutral Sphingomyelinases in Neurological Pathologies.
Respiratory Insufficiency
Staphylococcal alpha-toxin-induced vascular leakage in isolated perfused rabbit lungs.
Topical application of phosphatidyl-inositol-3,5-bisphosphate for acute lung injury in neonatal swine.
Retinal Degeneration
Lack of Acid Sphingomyelinase Induces Age-Related Retinal Degeneration.
Retinitis Pigmentosa
The plasma peptides of Alzheimer's disease.
Retinoblastoma
Neutral sphingomyelinase-2 mediates growth arrest by retinoic acid through modulation of ribosomal S6 kinase.
Role for mammalian neutral sphingomyelinase 2 in confluence-induced growth arrest of MCF7 cells.
Rhabdomyosarcoma
Ceramide-induced G2 arrest in rhabdomyosarcoma (RMS) cells requires p21Cip1/Waf1 induction and is prevented by MDM2 overexpression.
Rubella
Comparative study of immune status to infectious agents in elderly patients with multiple myeloma, Waldenstrom's macroglobulinemia, and monoclonal gammopathy of undetermined significance.
Schistosomiasis
In vitro and in vivo activities of arachidonic acid against Schistosoma mansoni and Schistosoma haematobium.
Novel therapeutic and prevention approaches for schistosomiasis: review.
Sea-Blue Histiocyte Syndrome
Biochemical, genetic and ultrastructural study of a family with the sea-blue histiocyte syndrome/chronic non-neuronopathic Niemann-Pick disease.
Partial sphingomyelinase deficiency with sea-blue histiocytosis and neurovisceral dysfunction.
Sphingomyelinase activity levels in human peripheral blood leukocytes, using [3H]sphingomyelin as substrate: study of heterozygotes and homozygotes for Niemann-Pick disease variants.
Seizures
A novel non-transcriptional pathway mediates the proconvulsive effects of interleukin-1beta.
Expression profiles of hippocampal regenerative sprouting-related genes and their regulation by E-64d in a developmental rat model of penicillin-induced recurrent epilepticus.
Long-term expression of metabolism-associated genes in the rat hippocampus following recurrent neonatal seizures and its regulation by melatonin.
Staphylococcal alpha toxin induced changes in the electroencephalogram of the rat.
Sepsis
A novel visible range FRET probe for monitoring acid sphingomyelinase activity in living cells.
Acid Sphingomyelinase Inhibition Prevents Development of Sepsis Sequelae in the Murine Liver.
Acid sphingomyelinase inhibition protects mice from lung edema and lethal Staphylococcus aureus sepsis.
Acid Sphingomyelinase Inhibition Stabilizes Hepatic Ceramide Content and Improves Hepatic Biotransformation Capacity in a Murine Model of Polymicrobial Sepsis.
Acid sphingomyelinase promotes endothelial stress response in systemic inflammation and sepsis.
Activation of acid sphingomyelinase and its inhibition by the nitric oxide/cyclic guanosine 3',5'-monophosphate pathway: key events in Escherichia coli-elicited apoptosis of dendritic cells.
Adjustment of Dysregulated Ceramide Metabolism in a Murine Model of Sepsis-Induced Cardiac Dysfunction.
Alternative splicing of SMPD1 in human sepsis.
APOPTOSIS CONTRIBUTES TO SEPTIC CARDIOMYOPATHY AND IS IMPROVED BY SIMVASTATIN THERAPY.
Apoptosis contributes to septic cardiomyopathy and is improved by simvastatin therapy.
Bacillus cereus sphingomyelinase recognizes ganglioside GM3.
Clostridium septicum myonecrosis presenting as a parapharyngeal abscess in a patient with aplastic anemia.
Decreased plasma phospholipid concentrations and increased acid sphingomyelinase activity are accurate biomarkers for community-acquired pneumonia.
Group B streptococcal beta-hemolysin induces mortality and liver injury in experimental sepsis.
Hyperresponsiveness of mice deficient in plasma-secreted sphingomyelinase reveals its pivotal role in early phase of host response.
Multimechanistic Monoclonal Antibodies (MAbs) Targeting Staphylococcus aureus Alpha-Toxin and Clumping Factor A: Activity and Efficacy Comparisons of a MAb Combination and an Engineered Bispecific Antibody Approach.
Programmed cellular necrosis mediated by the pore-forming alpha-toxin from Clostridium septicum.
Reevaluation of Lung Injury in TNF-Induced Shock: The Role of the Acid Sphingomyelinase.
Role of acid sphingomyelinase and IL-6 as mediators of endotoxin-induced pulmonary vascular dysfunction.
Role of increased sphingomyelinase activity in apoptosis and organ failure of patients with severe sepsis.
Role of sphingomyelinase in infectious diseases caused by Bacillus cereus.
Sphingomyelinase stimulates oxidant signaling to weaken skeletal muscle and promote fatigue.
Staphylococcus aureus ?-Toxin Mutants Are Defective in Biofilm Ligase and Sphingomyelinase Activity, and Causation of Infective Endocarditis/Sepsis.
Staphylococcus aureus ?-toxin Production is Common in Strains with the ?-toxin Gene Inactivated by Bacteriophage.
The role of antibodies against alpha-toxin and teichoic acid in the diagnosis of staphylococcal infections.
The role of sphingolipids in respiratory disease.
[Effect of lincomycin and staphylococcal vaccine on the course of experimental staphylococcal sepsis]
Severe Acute Respiratory Syndrome
Differential role of sphingomyelin in influenza virus, rhinovirus and SARS-CoV-2 infection of Calu-3 cells.
Pharmacological Inhibition of Acid Sphingomyelinase Prevents Uptake of SARS-CoV-2 by Epithelial Cells.
Shock, Septic
ADRENOMEDULLIN REDUCES INTESTINAL EPITHELIAL PERMEABILITY IN VIVO AND IN VITRO.
Antibody levels against eleven Staphylococcus aureus antigens in a healthy population.
Cloning, characterization, and sequencing of an accessory gene regulator (agr) in Staphylococcus aureus.
Clostridial glucosylating toxins enter cells via clathrin-mediated endocytosis.
Cryptic alpha-toxin gene in toxic shock syndrome and septicaemia strains of Staphylococcus aureus.
Cytolysins augment superantigen penetration of stratified mucosa.
Geographical variation in the presence of genes encoding superantigenic exotoxins and beta-hemolysin among Staphylococcus aureus isolated from bovine mastitis in Europe and USA.
IgG4 subclass-specific responses to Staphylococcus aureus antigens shed new light on host-pathogen interaction.
Rapid isolation and identification of staphylococcal exoproteins by reverse phase capillary high performance liquid chromatography-electrospray ionization mass spectrometry.
Reduction in Staphylococcus aureus Growth and Exotoxin Production and in Vaginal Interleukin 8 Levels Due to Glycerol Monolaurate in Tampons.
Staphylococcal alpha-toxin provokes coronary vasoconstriction and loss in myocardial contractility in perfused rat hearts: role of thromboxane generation.
Staphylococcal toxins and protein A differentially induce cytotoxicity and release of tumor necrosis factor-alpha from human keratinocytes.
Synergism of Candida albicans and delta toxin producing Staphylococcus aureus on mouse mortality and morbidity: protection by indomethacin.
The role of virulence factors in the outcome of staphylococcal peritonitis in CAPD patients.
Shwartzman Phenomenon
Different pathways leading to cutaneous leukocytoclastic vasculitis in mice.
Sinusitis
Effects of beta-toxin of Staphylococcus aureus on ciliary activity of nasal epithelial cells.
Small Cell Lung Carcinoma
Synchronous Bilateral Lung Cancer With Discordant Histology.
Soft Tissue Infections
Anti-alpha-toxin monoclonal antibody and antibiotic combination therapy improves disease outcome and accelerates healing in a Staphylococcus aureus dermonecrosis model.
Sphingolipidoses
A novel association between angiokeratoma corporis diffusum and acid sphingomyelinase deficiency.
A novel SMPD1 mutation in two Chinese sibling patients with type B Niemann-Pick disease.
Apoptosis and signalling in acid sphingomyelinase deficient cells.
My journey into the world of sphingolipids and sphingolipidoses.
sphingomyelin phosphodiesterase deficiency
A family with visceral course of Niemann-Pick disease, macular halo syndrome and low sphingomyelin degradation rate.
A new variant of sphingomyelinase deficiency (Niemann-Pick): visceromegaly, minimal neurological lesions and low in vivo degradation rate of sphingomyelin.
A novel association between angiokeratoma corporis diffusum and acid sphingomyelinase deficiency.
AAV8-mediated hepatic expression of acid sphingomyelinase corrects the metabolic defect in the visceral organs of a mouse model of Niemann-Pick disease.
Abrogation of early apoptosis does not alter late inhibition of hippocampal neurogenesis after irradiation.
Acid sphingomyelinase (aSMase) deficiency leads to abnormal microglia behavior and disturbed retinal function.
Acid sphingomyelinase deficiency (Niemann?Pick disease Type B) as an inflammatory disease.
Acid Sphingomyelinase Deficiency Ameliorates Farber Disease.
Acid sphingomyelinase deficiency attenuates bleomycin-induced lung inflammation and fibrosis in mice.
Acid sphingomyelinase deficiency contributes to resistance of scleroderma fibroblasts to Fas-mediated apoptosis.
Acid sphingomyelinase deficiency does not protect from graft-versus-host disease in transplant recipients with Niemann-Pick disease.
Acid sphingomyelinase deficiency enhances myelin repair after acute and chronic demyelination.
Acid sphingomyelinase deficiency exacerbates LPS-induced experimental periodontitis.
Acid sphingomyelinase deficiency in Beckwith Wiedemann syndrome.
Acid sphingomyelinase deficiency in Western diet-fed mice protects against adipocyte hypertrophy and diet-induced liver steatosis.
Acid sphingomyelinase deficiency increases susceptibility to fatal alphavirus encephalomyelitis.
Acid Sphingomyelinase Deficiency Prevents Diet-induced Hepatic Triacylglycerol Accumulation and Hyperglycemia in Mice.
Acid sphingomyelinase deficiency protects from cisplatin-induced gastrointestinal damage.
Acid sphingomyelinase deficiency protects mitochondria and improves function recovery after brain injury.
Acid sphingomyelinase deficiency. Phenotype variability with prevalence of intermediate phenotype in a series of twenty-five Czech and Slovak patients. A multi-approach study.
Acid sphingomyelinase deficiency: Cardiac dysfunction and characteristic findings of the coronary arteries.
Acid sphingomyelinase deficiency: prevalence and characterization of an intermediate phenotype of Niemann-Pick disease.
Acid Sphingomyelinase Inhibition Prevents Development of Sepsis Sequelae in the Murine Liver.
Acid sphingomyelinase possesses a domain homologous to its activator proteins: saposins B and D.
Adrenal changes in Niemann-Pick disease: differences between sphingomyelinase deficiency and type C.
Adult Niemann-Pick disease masquerading as sea blue histiocyte syndrome: report of a case confirmed by lipid analysis and enzyme assays.
Adult sphingomyelinase deficiency: report of 2 patients who initially presented with psychiatric disorders.
Altered Lipid Metabolism in Brain Injury and Disorders.
Altered lipid metabolism in brain injury and disorders.
Altered Macrophage Function Associated with Crystalline Lung Inflammation in Acid Sphingomyelinase Deficiency.
An animal model of human acid sphingomyelinase deficiency (Niemann-Pick disease) and the study of its enzyme replacement (the Japan Society of Human Genetics award lecture).
An Early-Onset Neuronopathic Form of Acid Sphingomyelinase Deficiency: A SMPD1 p.C133Y Mutation in the Saposin Domain of Acid Sphingomyelinase.
Application of a glycinated bile acid biomarker for diagnosis and assessment of response to treatment in Niemann-pick disease type C1.
Autopsy pathology of infantile neurovisceral ASMD (Niemann-Pick Disease type A): Clinicopathologic correlations of a case report.
Bax and Bak do not exhibit functional redundancy in mediating radiation-induced endothelial apoptosis in the intestinal mucosa.
Biochemical and imaging parameters in acid sphingomyelinase deficiency: Potential utility as biomarkers.
Burden of Illness in Acid Sphingomyelinase Deficiency: A Retrospective Chart Review of 100 Patients.
Case Report of Gastrointestinal Bleeding in an Adult with Chronic Visceral Acid Sphingomyelinase Deficiency.
Cause of death in patients with chronic visceral and chronic neurovisceral acid sphingomyelinase deficiency (Niemann-Pick disease type B and B variant): Literature review and report of new cases.
Chronic Niemann-Pick disease with sphingomyelinase deficiency in two brothers with mental retardation.
Chronic visceral acid sphingomyelinase deficiency (Niemann-Pick disease type B) in 16 Polish patients: long-term follow-up.
Clearance of Hepatic Sphingomyelin by Olipudase Alfa Is Associated With Improvement in Lipid Profiles in Acid Sphingomyelinase Deficiency.
Clinical relevance of endpoints in clinical trials for acid sphingomyelinase deficiency enzyme replacement therapy.
Combined Emphysema and Interstitial Lung Disease as a Rare Presentation of Pulmonary Involvement in a Patient with Chronic Visceral Acid Sphingomyelinase Deficiency (Niemann-Pick Disease Type B).
Complex kinetics of bis(4-methylumbelliferyl)phosphate and hexadecanoyl(nitrophenyl)phosphorylcholine hydrolysis by purified sphingomyelinase in the presence of Triton X-100.
Consensus recommendation for a diagnostic guideline for acid sphingomyelinase deficiency.
Correction of sphingomyelinase deficiency in Niemann-Pick type C fibroblasts by removal of lipoprotein fraction from culture media.
Corrigendum to "Cause of death in patients with chronic visceral and chronic neurovisceral acid sphingomyelinase deficiency (Niemann-Pick disease type B and B variant): Literature review and report of new cases" [Mol. Genet. Metab. 118 (2016) 206-213].
Critical assessment of chitotriosidase analysis in the rational laboratory diagnosis of children with Gaucher disease and Niemann-Pick disease type A/B and C.
Deficiency of phospholipase C acting on phosphatidylglycerol in Niemann-Pick disease.
Deficiency of taurocholate-dependent phospholipase C acting on phosphatidylcholine in Niemann-Pick disease.
Deposition of lipopigment--a new feature of human splenic sinus endothelium (SSE). Ultrastructural and histochemical study.
Disease manifestations and burden of illness in patients with acid sphingomyelinase deficiency (ASMD).
Dual diagnosis of Ochoa syndrome and Niemann-Pick disease type B in a consanguineous family.
Effect of dimethylsulfoxide on sphingomyelinase activity and cholesterol metabolism in Niemann-Pick type C fibroblasts.
Effect of host cell lipid metabolism on alphavirus replication, virion morphogenesis, and infectivity.
Effects of acid sphingomyelinase deficiency on male germ cell development and programmed cell death.
Effects of dimethylsulfoxide on sphingomyelinase in cultured human fibroblasts and correction of sphingomyelinase deficiency in fibroblasts from Niemann-Pick patients.
Enhanced Delivery and Effects of Acid Sphingomyelinase by ICAM-1-Targeted Nanocarriers in Type B Niemann-Pick Disease Mice.
Functional characterization of novel variants in SMPD1 in Indian patients with acid sphingomyelinase deficiency.
Gelastic cataplexy in Niemann-Pick disease group C and related variants without generalized sphingomyelinase deficiency.
Homozygous pArg610del Mutation Unusually Associated With Severe Delay of Growth in 2 Acid Sphingomyelinase Deficiency-affected Sibs.
Homozygous Smpd1 deficiency aggravates brain ischemia/ reperfusion injury by mechanisms involving polymorphonuclear neutrophils, whereas heterozygous Smpd1 deficiency protects against mild focal cerebral ischemia.
Human acid sphingomyelinase.
Identification and biochemical characterization of an acid sphingomyelinase-like protein from the bacterial plant pathogen Ralstonia solanacearum that hydrolyzes ATP to AMP but not sphingomyelin to ceramide.
Identification of a distinct mutation spectrum in the SMPD1 gene of Chinese patients with acid sphingomyelinase-deficient Niemann-Pick disease.
Increased liver tumor formation in neutral sphingomyelinase-2 deficient mice.
Inhibition of fatty acid amide hydrolase prevents pathology in neurovisceral acid sphingomyelinase deficiency by rescuing defective endocannabinoid signaling.
Interstitial lung disease in lysosomal storage disorders.
Isolation of Alpha-Toxin-Deficient Clostridium perfringens Type F from Sewage Influents and Effluents.
Ito cells in lysosomal storage disorders. An ultrastructural study.
Limited benefits of presymptomatic cord blood transplantation in neurovisceral acid sphingomyelinase deficiency (ASMD) intermediate type.
Lipid abnormalities in children with types A and B Niemann Pick disease.
Liver and skin histopathology in adults with acid sphingomyelinase deficiency (Niemann-Pick disease type B).
Local regulation of tooth mineralization by sphingomyelin phosphodiesterase 3.
Long-term efficacy of olipudase alfa in adults with acid sphingomyelinase deficiency (ASMD): Further clearance of hepatic sphingomyelin is associated with additional improvements in pro- and anti-atherogenic lipid profiles after 42 months of treatment.
Macula halo syndrome. Variant of Niemann-Pick disease.
Mitochondrial biogenesis is transcriptionally repressed in lysosomal lipid storage diseases.
Molecular analysis of the acid sphingomyelinase deficiency in a family with an intermediate form of Niemann-Pick disease.
Molecular basis of acid sphingomyelinase deficiency in a patient with Niemann-Pick disease type A.
MRI in an unusually protracted neuronopathic variant of acid sphingomyelinase deficiency.
Neutral Sphingomyelinase (SMPD3) Deficiency Causes a Novel Form of Chondrodysplasia and Dwarfism That Is Rescued by Col2A1-Driven smpd3 Transgene Expression.
Neutral sphingomyelinase (SMPD3) deficiency causes a novel form of chondrodysplasia and dwarfism that is rescued by Col2A1-driven smpd3 transgene expression.
Neutral sphingomyelinase (SMPD3) deficiency disrupts the Golgi secretory pathway and causes growth inhibition.
Neutral sphingomyelinase 1 deficiency in the mouse causes no lipid storage disease.
Neutral Sphingomyelinase 2 (SMPD3) Deficiency in Mice Causes Chondrodysplasia with Unimpaired Skeletal Mineralization.
Neutral sphingomyelinase 2 deficiency increases hyaluronan synthesis by up-regulation of hyaluronan Synthase 2 through decreased ceramide production and activation of Akt.
Neutral sphingomyelinase 2 deficiency is associated with lung anomalies similar to emphysema.
Neutral Sphingomyelinase-2 Deficiency Ameliorates Alzheimer's Disease Pathology and Improves Cognition in the 5XFAD Mouse.
New findings in pathology of storage.
Niemann-Pick disease (variation in the sphingomyelinase deficient group). Neurovisceral phenotype (A) with an abnormally protracted clinical course and variable expression of neurological symptomatology in three siblings.
Niemann-Pick disease and juvenile xanthogranuloma. Are they related?
Niemann-Pick disease type A and B are clinically but also enzymatically heterogeneous: pitfall in the laboratory diagnosis of sphingomyelinase deficiency associated with the mutation Q292 K.
Niemann-Pick Disease versus acid sphingomyelinase deficiency.
Niemann-Pick disease.
Niemann-Pick disease: lipid storage in bone marrow macrophages.
Niemann-Pick type C disease: Novel NPC1 mutations and characterization of the concomitant acid sphingomyelinase deficiency.
Niemann-Pick type C disease: The atypical sphingolipidosis.
Nonclinical safety assessment of recombinant human acid sphingomyelinase (rhASM) for the treatment of acid sphingomyelinase deficiency:the utility of animal models of disease in the toxicological evaluation of potential therapeutics.
Novel mutations in the SMPD1 gene in Jordanian children with Acid sphingomyelinase deficiency (Niemann-Pick types A and B).
Ocular manifestations of adult Niemann-Pick disease: a case report.
Ocular pathology of infantile Niemann-Pick disease. Study of fetus of 23 weeks' gestation.
Olipudase alfa for treatment of acid sphingomyelinase deficiency (ASMD): safety and efficacy in adults treated for 30 months.
One-year results of a clinical trial of olipudase alfa enzyme replacement therapy in pediatric patients with acid sphingomyelinase deficiency.
Optimization of a histopathological biomarker for sphingomyelin accumulation in Acid sphingomyelinase deficiency.
Oral, dental, and craniofacial features in chronic acid sphingomyelinase deficiency.
Partial sphingomyelinase deficiency with sea-blue histiocytosis and neurovisceral dysfunction.
Pathogenesis of one variant of sea-blue histiocytosis.
Periodontal condition and treatment in a patient with rare systemic condition: A case report for acid sphingomyelinase deficiency.
Peripheral neuropathy in type A Niemann-Pick disease. A morphological study.
Phenotypic and genetic heterogeneity in Niemann-Pick disease type C: current knowledge and practical implications.
Plasma lyso-sphingomyelin levels are positively associated with clinical severity in acid sphingomyelinase deficiency.
Polyneuropathy in feline Niemann-Pick disease.
Prospective study of the natural history of chronic acid sphingomyelinase deficiency in children and adults: eleven years of observation.
Quantitation of plasmatic lysosphingomyelin and lysosphingomyelin-509 for differential screening of Niemann-Pick A/B and C diseases.
Quantitative Systems Pharmacology Modeling of Acid Sphingomyelinase Deficiency and the Enzyme Replacement Therapy Olipudase Alfa Is an Innovative Tool for Linking Pathophysiology and Pharmacology.
Radiation-induced apoptosis of endothelial cells in the murine central nervous system: protection by fibroblast growth factor and sphingomyelinase deficiency.
Rapid screening for lipid storage disorders using biochemical markers. Expert center data and review of the literature.
Recommendations for clinical monitoring of patients with acid sphingomyelinase deficiency (ASMD).
Reconstruction of the Cytokine Signaling in Lysosomal Storage Diseases by Literature Mining and Network Analysis.
Regulation of Chlamydomonas flagella and ependymal cell motile cilia by ceramide-mediated translocation of GSK3.
Reliable Assay of Acid Sphingomyelinase Deficiency with the Mutation Q292K by Tandem Mass Spectrometry.
Respiratory impairment in Niemann-Pick B disease: Two case reports and review for the pulmonologist.
Role of acidic sphingomyelinase in Fas/CD95-mediated cell death.
Role of SMPD3 during Bone Fracture Healing and Regulation of Its Expression.
Serum Zn(2+)-stimulated sphingomyelinase deficiency in type B Niemann-Pick disease.
Seven novel mutations of the SMPD1 gene in four Chinese patients with Niemann-Pick disease type A and prenatal diagnosis for four fetuses.
Signaling in and regulation of ionizing radiation-induced apoptosis in endothelial cells.
Skeletal manifestations in pediatric and adult patients with Niemann Pick disease type B.
SMPD3 deficiency perturbs neuronal proteostasis and causes progressive cognitive impairment.
SMPDL3b modulates insulin receptor signaling in diabetic kidney disease.
Sphingolipid metabolism and its role in the skeletal tissues.
Sphingomyelin lipidosis (Niemann-Pick disease) in a juvenile raccoon (Procyon lotor).
Sphingomyelinase activities of various human tissues in control subjects and in Niemann-Pick disease - development and evaluation of a microprocedure.
Sphingomyelinase deficiency (Niemann-Pick disease) in a Hereford calf.
Splenic histiocytosis in idiopathic thrombocytopenic purpura: a relative sphingomyelinase deficiency?
Structural and functional analysis of the ASM p.Ala359Asp mutant that causes acid sphingomyelinase deficiency.
Subclinical course of adult visceral Niemann-Pick type C1 disease. A rare or underdiagnosed disorder?
Successful within-patient dose escalation of olipudase alfa in acid sphingomyelinase deficiency.
Synthesis of pyrenesulfonylamido-sphingomyelin and its use as substrate for determining sphingomyelinase activity and diagnosing Niemann-Pick disease.
The IL-1 receptor signaling pathway.
The impact of biomarkers analysis in the diagnosis of Niemann-Pick C disease and acid sphingomyelinase deficiency.
The natural history of type B Niemann-Pick disease: results from a 10-year longitudinal study.
Treatment of sphingomyelinase deficiency by repeated implantations of amniotic epithelial cells.
Tricyclic antidepressants induce sphingomyelinase deficiency in fibroblast and neuroblastoma cell cultures.
Twenty- five years of biochemical diagnosis of Gaucher disease: the Egyptian experience.
Two novel mutations in patients with atypical phenotypes of acid sphingomyelinase deficiency.
Two Siblings with Niemann-Pick Disease (NPD) Type B: Clinical Findings and Novel Mutations of the Acid Sphingomyelinase Gene.
Uptake and metabolism of radioactively labeled sphingomyelin in cultured skin fibroblasts from controls and patients with Niemann-Pick disease and other lysosomal storage diseases.
[Acid sphingomyelinase deficiency (Niemann-Pick disease type B) in adulthood: A retrospective multicentric study of 28 adult cases].
[Acid sphingomyelinase deficiency and spleen trauma: Splenectomy or not splenectomy?]
[Activators for sphingohydrolases and the nature of the sphingomyelinase deficiency in Niemann-Pick disease types A, B and C (author's transl)]
[Lipidosis with vertical gaze palsy, macular degeneration, and sphingomyelinase deficiency]
[Lysosomal sphingomyelinase deficiency: spectrum of phenotypes in Czech and Slovak patients]
[Niemann-Pick disease revealed late by miliary tuberculosis: diagnosis by leukocyte determination of sphingomyelinase deficiency]
[Perhexiline maleate induces sphingomyelinase deficiency in man]
[Sea-blue histiocyte syndrome (author's transl)]
[Sphingomyelinase deficiency (Niemann-Pick) type A. A new variant with an unusually prolonged course]
[Study of thesaurismosis induced by perhexiline maleate. Confirmation of experimental data]
Spinal Cord Injuries
Interactions between neural membrane glycerophospholipid and sphingolipid mediators: a recipe for neural cell survival or suicide.
Squamous Cell Carcinoma of Head and Neck
Acid sphingomyelinase activity as an indicator of the cell stress in HPV-positive and HPV-negative head and neck squamous cell carcinoma.
Sphingomyelin phosphodiesterase 3 methylation and silencing in oral squamous cell carcinoma results in increased migration and invasion and altered stress response.
Staphylococcal Infections
Acute bacterial skin and skin structure infection: Critical role of alpha-toxin and protective effects of its neutralization by a human antibody.
Biology and clinical significance of peptidoglycan antibody response in staphylococcal infections.
Effect of 2 Emulsion-Based Adjuvants on the Structure and Thermal Stability of Staphylococcus aureus Alpha-Toxin.
Effects of amoxicillin, gentamicin, and moxifloxacin on the hemolytic activity of Staphylococcus aureus in vitro and in vivo.
Incorporation of staphylococcal alpha-toxin in glutaraldehyde fixed erythrocytes.
Interferons increase cell resistance to Staphylococcal alpha-toxin.
Lipase versus teichoic acid and alpha-toxin as antigen in an enzyme immunoassay for serological diagnosis of Staphylococcus aureus infections.
Local delivery of soluble interleukin-6 receptors to improve the outcome of alpha-toxin producing Staphylococcus aureus infection in mice.
Regulated antisense RNA eliminates alpha-toxin virulence in Staphylococcus aureus infection.
Regulation of Staphylococcus aureus Infection of Macrophages by CD44, Reactive Oxygen Species, and Acid Sphingomyelinase.
Release of interleukin-1 beta associated with potent cytocidal action of staphylococcal alpha-toxin on human monocytes.
Serological diagnosis of deep Staphylococcus aureus infections by enzyme-linked immunosorbent assay (ELISA) for staphylococcal hemolysins and teichoic acid.
Stimulation of PAF-synthesis in pulmonary artery endothelial cells by Staphylococcus aureus alpha-toxin.
The role of antibodies against alpha-toxin and teichoic acid in the diagnosis of staphylococcal infections.
[Characteristics of a staphylococcal infection in guinea pigs with delayed hypersensitivity to staphylococcal alpha-toxin]
[Development of approaches to studying the structural and functional organization of protein molecules aboard the International space station]
Staphylococcal Scalded Skin Syndrome
Staphylococcal scalded skin syndrome. II. Serum level of anti exfoliatin and anti alpha-toxin in patients with staphylococcal scalded skin syndrome or bullous impetigo.
Starvation
Activation of neutral sphingomyelinase 2 by starvation induces cell-protective autophagy via an increase in Golgi-localized ceramide.
Hyaluronan synthase-2 upregulation protects smpd3-deficient fibroblasts against cell death induced by nutrient deprivation, but not against apoptosis evoked by oxidized LDL.
[Functional state of a sphingomyeline cycle and free radical lipid oxidation activity of a rat's liver during different phases of starvation].
sterol esterase deficiency
Application of a glycinated bile acid biomarker for diagnosis and assessment of response to treatment in Niemann-pick disease type C1.
Stomach Neoplasms
Acid Sphingomyelinase Contributes to Evodiamine-Induced Apoptosis in Human Gastric Cancer SGC-7901 Cells.
Gentamicin Targets Acid Sphingomyelinase in Cancer: The Case of the Human Gastric Cancer NCI-N87 Cells.
Stroke
Changes in the cerebrospinal fluid lipid profile following subarachnoid hemorrhage in a closed cranium model: Correlations to cerebral vasospasm, neuronal cell death and Interleukin-6 synthesis. A pilot study.
Interactions between neural membrane glycerophospholipid and sphingolipid mediators: a recipe for neural cell survival or suicide.
Neonatal stroke enhances interaction of microglia-derived extracellular vesicles with microglial cells.
Neutral sphingomyelinase: past, present and future.
Pivotal role for acidic sphingomyelinase in cerebral ischemia-induced ceramide and cytokine production, and neuronal apoptosis.
Sphingolipids in atherosclerosis and vascular biology.
Sphingomyelinase and ceramide analogs induce vasoconstriction and leukocyte-endothelial interactions in cerebral venules in the intact rat brain: Insight into mechanisms and possible relation to brain injury and stroke.
The Acid Sphingomyelinase/ Ceramide System as Target for Ischemic Stroke Therapies.
Surgical Wound Infection
Efficacy of a Multimechanistic Monoclonal Antibody Combination against Staphylococcus aureus Surgical Site Infections in Mice.
Synucleinopathies
Lysosomal enzyme activities as possible CSF biomarkers of synucleinopathies.
Systemic Vasculitis
Elevated active secretory sphingomyelinase in antineutrophil cytoplasmic antibody-associated primary systemic vasculitis.
Tay-Sachs Disease
Bi-functional IgG-lysosomal enzyme fusion proteins for brain drug delivery.
Tendinopathy
Selected Clinical Features Fail to Predict Inflammatory Gene Expressions for TNF-?, TNFR1, NSMAF, Casp3 and IL-8 in Tendons of Patients with Rotator Cuff Tendinopathy.
Tetanus
Bacterial toxins: friends or foes?
Comparative study of immune status to infectious agents in elderly patients with multiple myeloma, Waldenstrom's macroglobulinemia, and monoclonal gammopathy of undetermined significance.
Distinct targets for tetanus and botulinum A neurotoxins within the signal transducing pathway in chromaffin cells.
Multiple effects of alpha-toxins on the nicotinic acetylcholine receptor.
Probiotics stimulate production of natural antibodies in chickens.
Protein antigens increase the protective efficacy of a capsule-based vaccine against Staphylococcus aureus in a rat model of osteomyelitis.
Some general rules governing fate and action of polypeptide and protein drugs, as derived from investigations with staphylococcal alpha-toxin, tetanus toxin, and so-calledd crotoxin.
Thrombocytopenia
Acute Gaucher Disease-Like Condition in an Indian Infant with a Novel Biallelic Mutation in the Prosaposin Gene.
Thrombophilia
SARS-CoV-2 infection induces the activation of tissue factor-mediated coagulation via activation of acid sphingomyelinase.
The Role of Chemoprophylactic Agents in Modulating Platelet Aggregability After Traumatic Brain Injury.
Thrombosis
Acid sphingomyelinase regulates platelet cell membrane scrambling, secretion, and thrombus formation.
Identification of key lipids critical for platelet activation by comprehensive analysis of the platelet lipidome.
Toxemia
Relation of in vitro inhibition by chelates of Clostridium perfringens alpha-toxin to their ability to protect against experimental toxemia.
STUDIES OF THE EFFECT OF ALDOSTERONE IN THE EXPERIMENTAL TOXEMIA WITH THE ALPHA-TOXIN OF CLOSTRIDIUM WELCHII.
Toxic Optic Neuropathy
Specific roles of alpha-toxin and beta-toxin during Staphylococcus aureus corneal infection.
Transfusion-Related Acute Lung Injury
Platelet extracellular vesicles mediate transfusion-related acute lung injury by imbalancing the sphingolipid rheostat.
Tremor
Staphylococcal alpha toxin induced changes in the electroencephalogram of the rat.
Tuberculosis
A novel class of microbial phosphocholine-specific phospholipases C.
An acidic sphingomyelinase Type C activity from Mycobacterium tuberculosis.
Extracellular Sphingomyelinase Rv0888 of Mycobacterium tuberculosis Contributes to Pathological Lung Injury of Mycobacterium smegmatis in Mice via Inducing Formation of Neutrophil Extracellular Traps.
Identification of acidic, alkaline, and neutral sphingomyelinase activities in Mycobacterium tuberculosis.
Inhibition of neutral sphingomyelinase protects mice against systemic tuberculosis.
Sphingomyelinase D/ceramide 1-phosphate in cell survival and inflammation.
Tuberculosis, Miliary
[Niemann-Pick disease revealed late by miliary tuberculosis: diagnosis by leukocyte determination of sphingomyelinase deficiency]
Urinary Tract Infections
Hemolysin and K antigens in relation to serotype and hemagglutination type of Escherichia coli isolated from extraintestinal infections.
Uterine Cervicitis
Cervicovaginal Inflammatory Cytokines and Sphingomyelinase in Women With and Without Bacterial Vaginosis.
Vaccinia
Recombinant vaccinia viruses protect against Clostridium perfringens alpha-toxin.
Vaginitis
Cervicovaginal Inflammatory Cytokines and Sphingomyelinase in Women With and Without Bacterial Vaginosis.
Vaginosis, Bacterial
Cervicovaginal Inflammatory Cytokines and Sphingomyelinase in Women With and Without Bacterial Vaginosis.
Vascular Calcification
Acid sphingomyelinase promotes SGK1-dependent vascular calcification.
Ceramide mediates Ox-LDL-induced human vascular smooth muscle cell calcification via p38 mitogen-activated protein kinase signaling.
Vascular System Injuries
Chemotherapy-induced acute vascular injury involves intracellular generation of ROS via activation of the acid sphingomyelinase pathway.
Enhancement of Soft Tissue Sarcoma Response to Gemcitabine through Timed Administration of a Short-Acting Anti-Angiogenic Agent.
Vesicular Stomatitis
Pharmacological Inhibition of Acid Sphingomyelinase Prevents Uptake of SARS-CoV-2 by Epithelial Cells.
Virus Diseases
Ceramide and cell death receptor clustering.
Depletion of Host and Viral Sphingomyelin Impairs Influenza Virus Infection.
Differential role of sphingomyelin in influenza virus, rhinovirus and SARS-CoV-2 infection of Calu-3 cells.
Oxidative Stress Triggers Ca-Dependent Lysosome Trafficking and Activation of Acid Sphingomyelinase.
SARS-CoV-2 infection induces the activation of tissue factor-mediated coagulation via activation of acid sphingomyelinase.
Whooping Cough
Aerolysin induces G-protein activation and Ca2+ release from intracellular stores in human granulocytes.
Hemolysis induced by Bacillus cereus sphingomyelinase.
Pertussis toxin promotes macrophage survival through inhibition of acid sphingomyelinase and activation of the phosphoinositide 3-kinase/protein kinase B pathway.
The relationship between the metabolism of sphingomyelin species and the hemolysis of sheep erythrocytes induced by Clostridium perfringens {alpha}-toxin.
The sphingomyelin/ceramide pathway is involved in ERK1/2 phosphorylation, cell proliferation, and uPAR overexpression induced by tissue-type plasminogen activator.
Wolman Disease
Application of a glycinated bile acid biomarker for diagnosis and assessment of response to treatment in Niemann-pick disease type C1.
Ito cells in lysosomal storage disorders. An ultrastructural study.
Wound Infection
Positive camp-phenomenon elicited by coagulase-negative staphylococci.
Virulence factors of Staphylococcus aureus in the pathogenesis of endocarditis. A comparative study of clinical isolates.