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Disease on EC 2.3.1.21 - carnitine O-palmitoyltransferase

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
6-phosphofructokinase deficiency
Abnormal oxidative metabolism and O2 transport in muscle phosphofructokinase deficiency.
Acidosis
Brief report: renal tubular acidosis in carnitine palmitoyltransferase type 1 deficiency.
Rate-dependent distal renal tubular acidosis and carnitine palmitoyltransferase I deficiency.
Acidosis, Renal Tubular
Brief report: renal tubular acidosis in carnitine palmitoyltransferase type 1 deficiency.
Rate-dependent distal renal tubular acidosis and carnitine palmitoyltransferase I deficiency.
Acute Kidney Injury
Acute renal failure due to carnitine palmitoyltransferase deficiency.
Acute renal failure due to carnitine palmitoyltransferase II deficiency.
An ignored cause of red urine in children: rhabdomyolysis due to carnitine palmitoyltransferase II (CPT-II) deficiency.
Carnitine palmitoyl transferase II deficiency in an adolescent presenting with rhabdomyolysis and acute renal failure.
Carnitine palmitoyltransferase deficiency: an underdiagnosed condition?
Carnitine Palmitoyltransferase II Deficiency (CPT II) Followed By Rhabdomyolysis and Acute Kidney Injury.
Carnitine palmitoyltransferase II deficiency due to a novel gene variant in a patient with rhabdomyolysis and ARF.
Exome-Based Rare-Variant Analyses in CKD.
Functional analysis of iPSC-derived myocytes from a patient with carnitine palmitoyltransferase II deficiency.
[Acute renal insufficiency caused by rhabdomyolysis due to deficiency of carnitine palmitoyltransferase]
[Mitochondrial activities of citrate synthase, carnitine palmitoyltransferase-1 and cytochrome C oxidase are increased during the apoptotic process in hepatocytes of a rat model of acute liver failure].
[Rhabdomyolysis caused by carnitine palmitoyltransferase deficiency. Prevention of acute kidney failure]
[Rhabdomyolysis due to muscle enzyme deficiencies]
acyl-coa dehydrogenase deficiency
Fatty Acid oxidation disorders in a chinese population in taiwan.
Follow-up of fatty acid ?-oxidation disorders in expanded newborn screening era.
In vivo stable isotope studies in three patients affected with mitochondrial fatty acid oxidation disorders: limited diagnostic use of 1-13C fatty acid breath test using bolus technique.
Therapeutic advances in the management of Pompe disease and other metabolic myopathies.
[Application of tandem mass spectrometry on the diagnosis of fatty acid oxidation disorders]
[Tandem mass spectrometry analysis and genetic diagnosis of neonates with fatty acid oxidation disorders in central and northern regions of Guangxi].
Adenocarcinoma
An indispensable role of CPT-1a to survive cancer cells during energy stress through rewiring cancer metabolism.
Alzheimer Disease
Carnitine acyltransferases are not changed in Alzheimer disease.
Carnitine palmitoyltransferase 1 (CPT1) alleviates oxidative stress and apoptosis of hippocampal neuron in response to beta-Amyloid peptide fragment A?25-35.
amp deaminase deficiency
Mutant carnitine palmitoyltransferase associated with myoadenylate deaminase deficiency in skeletal muscle.
Angina, Stable
Inhibition of long-chain fatty acid metabolism does not affect platelet aggregation responses.
Angina, Unstable
Inhibition of long-chain fatty acid metabolism does not affect platelet aggregation responses.
Aortic Valve Disease
Deciphering the gene expression profile of peroxisome proliferator-activated receptor signaling pathway in the left atria of patients with mitral regurgitation.
Asthma
Unmasking of carnitine palmitoyltransferase deficiency during an acute exacerbation of asthma complicated by rhabdomyolysis in a soldier.
Ataxia Telangiectasia
Resveratrol ameliorates high-fat diet-induced insulin resistance and fatty acid oxidation via ATM-AMPK axis in skeletal muscle.
Atherosclerosis
Carnitine palmitoyltransferase in patients with cardiac ischemia due to atherosclerotic coronary artery disease and in patients with idiopathic dilated cardiomyopathy.
Lutein Prevents High Fat Diet-Induced Atherosclerosis in ApoE-Deficient Mice by Inhibiting NADPH Oxidase and Increasing PPAR Expression.
[Effect of dachaihutang on expression of carnitine patmitoryl transferase-1 in vascular smooth muscle layer of atherosclerotic rabbits]
Azotemia
High-calorie diet partially ameliorates dysregulation of intrarenal lipid metabolism in remnant kidney.
Bradycardia
Lethal neonatal presentation of carnitine palmitoyltransferase I deficiency.
Brain Diseases
A Case of Recurrent Acute Encephalopathy with Febrile Convulsive Status Epilepticus with Carnitine Palmitoyltransferase II Variation.
Abbreviated half-lives and impaired fuel utilization in carnitine palmitoyltransferase II variant fibroblasts.
Acute necrotizing encephalopathy and a carnitine palmitoyltransferase 2 variant in an adult.
Acute severe encephalopathy related to human herpesvirus-6 infection in a patient with carnitine palmitoyltransferase 2 deficiency carrying thermolabile variants.
Bezafibrate upregulates carnitine palmitoyltransferase II expression and promotes mitochondrial energy crisis dissipation in fibroblasts of patients with influenza-associated encephalopathy.
Carnitine palmitoyl transferase II polymorphism is associated with multiple syndromes of acute encephalopathy with various infectious diseases.
Carnitine palmitoyltransferase 1C: From cognition to cancer.
Characterization of compound missense mutation and deletion of carnitine palmitoyltransferase II in a patient with adenovirus-associated encephalopathy.
Energy metabolic disorder is a major risk factor in severe influenza virus infection: Proposals for new therapeutic options based on animal model experiments.
Fatal viral infection-associated encephalopathy in two Chinese boys: a genetically determined risk factor of thermolabile carnitine palmitoyltransferase II variants.
Hyperlipidaemia due to carnitine palmitoyltransferase I deficiency.
Newborn screening for carnitine palmitoyltransferase II deficiency using (C16+C18:1)/C2: Evaluation of additional indices for adequate sensitivity and lower false-positivity.
Normal muscle CPT1 and CPT2 activities in hepatic presentation patients with CPT1 deficiency in fibroblasts. Tissue specific isoforms of CPT1?
Novel metabolic and molecular findings in hepatic carnitine palmitoyltransferase I deficiency.
Pandemic influenza A-associated acute necrotizing encephalopathy without neurologic sequelae.
Severe Human Herpesvirus 6-associated Encephalopathy in Three Children: Analysis of Cytokine Profiles and the Carnitine Palmitoyltransferase 2 Gene.
Severe Hyperammonemic Encephalopathy Requiring Dialysis Aggravated by Prolonged Fasting and Intermittent High Fat Load in a Ramadan Fasting Month in a Patient with CPTII Homozygous Mutation.
Thermal instability of compound variants of carnitine palmitoyltransferase II and impaired mitochondrial fuel utilization in influenza-associated encephalopathy.
Thermolabile CPT II variants and low blood ATP levels are closely related to severity of acute encephalopathy in Japanese children.
Thermolabile phenotype of carnitine palmitoyltransferase II variations as a predisposing factor for influenza-associated encephalopathy.
Thermolabile polymorphism of carnitine palmitoyltransferase 2: A genetic risk factor of overall acute encephalopathy.
Brain Edema
Thermal instability of compound variants of carnitine palmitoyltransferase II and impaired mitochondrial fuel utilization in influenza-associated encephalopathy.
Thermolabile CPT II variants and low blood ATP levels are closely related to severity of acute encephalopathy in Japanese children.
Brain Neoplasms
Poly-ion complex micelles effectively deliver CoA-conjugated CPT1A inhibitors to modulate lipid metabolism in brain cells.
Breast Neoplasms
Adipocyte lipolysis links obesity to breast cancer growth: adipocyte-derived fatty acids drive breast cancer cell proliferation and migration.
An integrated genomics approach identifies drivers of proliferation in luminal-subtype human breast cancer.
C75 increases peripheral energy utilization and fatty acid oxidation in diet-induced obesity.
Carnitine palmitoyl transferase 1A is a novel diagnostic and predictive biomarker for breast cancer.
Carnitine palmitoyl transferase-1A (CPT1A): a new tumor specific target in human breast cancer.
CPT1A and fatty acid ?-oxidation are essential for tumor cell growth and survival in hormone receptor-positive breast cancer.
CPT1A regulates breast cancer-associated lymphangiogenesis via VEGF signaling.
Establishing a relationship between prolactin and altered fatty acid ?-oxidation via carnitine palmitoyl transferase 1 in breast cancer cells.
Fatty acid oxidation is associated with proliferation and prognosis in breast and other cancers.
Fatty acid synthase inhibition in human breast cancer cells leads to malonyl-CoA-induced inhibition of fatty acid oxidation and cytotoxicity.
Fatty acid synthase inhibition triggers apoptosis during S phase in human cancer cells.
In Situ Metabolic Characterisation of Breast Cancer and Its Potential Impact on Therapy.
Influence of DMBA-induced mammary cancer on the liver CPT I, mit HMG-CoA synthase and PPARalpha mRNA expression in rats fed a low or high corn oil diet.
Mechanism of apoptosis induced by the inhibition of fatty acid synthase in breast cancer cells.
Molecular characterization, genomic structure and expression analysis of a gene (CATL1/CPT1C) encoding a third member of the human carnitine acyltransferase family.
Novel Inhibitors of Fatty Acid Synthase with Anticancer Activity.
carbamoyl-phosphate synthase (ammonia) deficiency
Targeted metabolomic analysis of plasma samples for the diagnosis of inherited metabolic disorders.
carboxypeptidase t deficiency
A Novel Mutation in CPT1A Resulting in Hepatic CPT Deficiency.
Biochemical evidence for heterozygosity in muscular carnitine palmitoyltransferase deficiency.
Central nervous system disorders and possible brain type carnitine palmitoyltransferase II deficiency.
Human liver mitochondrial carnitine palmitoyltransferase I: characterization of its cDNA and chromosomal localization and partial analysis of the gene.
Inhibition of carnitine palmitoyltransferase (CPT) by chlorpromazine in muscle of patients with CPT deficiency.
Inhibition of carnitine palmitoyltransferase in normal human skeletal muscle and in muscle of patients with carnitine palmitoyltransferase deficiency by long- and short-chain acylcarnitine and acyl-coenzyme A.
Limited trypsin proteolysis renders carnitine palmitoyltransferase insensitive to inhibition by malonyl-CoA in patients with muscle carnitine palmitoyltransferase deficiency.
Regulatory properties of a mutant carnitine palmitoyltransferase in human skeletal muscle.
Selective carnitine palmitoyltransferase deficiency in fibroblasts from a patient with muscle CPT deficiency.
Carcinogenesis
Carnitine palmitoyltransferase 1C contributes to progressive cellular senescence.
Carnitine palmitoyltransferase 1C regulates cancer cell senescence through mitochondria-associated metabolic reprograming.
Depletion of the novel p53-target gene carnitine palmitoyltransferase 1C delays tumor growth in the neurofibromatosis type I tumor model.
Development of a Genetic and Clinical Data-Based (GC) Risk Score for Predicting Survival of Hepatocellular Carcinoma Patients After Tumor Resection.
Targeting CPT1A enhances metabolic therapy in human melanoma cells with the BRAF V600E mutation.
Transcriptomic and Proteomic Analysis of Steatohepatitic Hepatocellular Carcinoma Reveals Novel Distinct Biologic Features.
Carcinoma
Carnitine palmitoyltransferase 1A functions to repress FoxO transcription factors to allow cell cycle progression in ovarian cancer.
Carnitine palmitoyltransferase 1C regulates cancer cell senescence through mitochondria-associated metabolic reprograming.
Carnitine palmitoyltransferase I in human carcinomas: a novel role in histone deacetylation?
Identification of CPT1A as a Prognostic Biomarker and Potential Therapeutic Target for Kidney Renal Clear Cell Carcinoma and Establishment of a Risk Signature of CPT1A-Related Genes.
Carcinoma, Hepatocellular
?-Tocotrienol attenuates triglyceride through effect on lipogenic gene expressions in mouse hepatocellular carcinoma Hepa 1-6.
Development of a Genetic and Clinical Data-Based (GC) Risk Score for Predicting Survival of Hepatocellular Carcinoma Patients After Tumor Resection.
Loss of response of carnitine palmitoyltransferase I to okadaic acid in transformed hepatic cells.
Metabolic profiling analysis upon acylcarnitines in tissues of hepatocellular carcinoma revealed the inhibited carnitine shuttle system caused by the downregulated carnitine palmitoyltransferase 2.
Non-transplant therapies for patients with hepatocellular carcinoma and Child-Pugh-Turcotte class B cirrhosis.
Positive regulation of hepatic carnitine palmitoyl transferase 1A (CPT1A) activities by soy isoflavones and L-carnitine.
Purple Tea and Its Extract Suppress Diet-induced Fat Accumulation in Mice and Human Subjects by Inhibiting Fat Absorption and Enhancing Hepatic Carnitine Palmitoyltransferase Expression.
Role of peroxisome proliferator-activated receptor alpha in the expression of hepatic fatty acid oxidation-related genes in chickens.
Simultaneously targeting SOAT1 and CPT1A ameliorates hepatocellular carcinoma by disrupting lipid homeostasis.
Transcriptomic and Proteomic Analysis of Steatohepatitic Hepatocellular Carcinoma Reveals Novel Distinct Biologic Features.
Carcinosarcoma
Carnitine palmitoyltransferase II activity is decreased in liver mitochondria of cachectic rats bearing the Walker 256 carcinosarcoma: effect of indomethacin treatment.
Cardiomegaly
Acute L-CPT1 Overexpression Recapitulates Reduced Palmitate Oxidation of Cardiac Hypertrophy.
Apigenin ameliorates hypertension-induced cardiac hypertrophy and down-regulates cardiac hypoxia inducible factor-l? in rats.
Carnitine palmitoyl transferase-I inhibition is not associated with cardiac hypertrophy in rats fed a high-fat diet.
Carnitine Palmitoyltransferase-1b (CPT1b) Deficiency Aggravates Pressure-Overload-Induced Cardiac Hypertrophy due to Lipotoxicity.
Comparison of the effects of carnitine palmitoyltransferase-1 and -2 inhibitors on rat heart hypertrophy.
Down-regulation of acyl-CoA oxidase gene expression and increased NF-kappaB activity in etomoxir-induced cardiac hypertrophy.
Etomoxir improves left ventricular performance of pressure-overloaded rat heart.
Hypoglycemic effects of a novel fatty acid oxidation inhibitor in rats and monkeys.
Improving characterization of hypertrophy-induced murine cardiac dysfunction using four-dimensional ultrasound-derived strain mapping.
Modification of subcellular organelles in pressure-overloaded heart by etomoxir, a carnitine palmitoyltransferase I inhibitor.
Octanoate is differentially metabolized in liver and muscle and fails to rescue cardiomyopathy in CPT2 deficiency.
Overnourishment during lactation induces metabolic and haemodynamic heart impairment during adulthood.
Peroxisome proliferator-activated receptor beta/delta activation inhibits hypertrophy in neonatal rat cardiomyocytes.
Transcribed Ultraconserved Regions, Uc.323, Ameliorates Cardiac Hypertrophy by Regulating the Transcription of CPT1b (Carnitine Palmitoyl transferase 1b).
Cardiomyopathies
Cardiac PPARalpha expression in patients with dilated cardiomyopathy.
Clinical and genetic characteristics of patients with fatty acid oxidation disorders identified by newborn screening.
Effect of L-carnitine supplementation on cardiac carnitine palmitoyltransferase activities and plasma carnitine concentrations in adriamycin-treated rats.
Lethal neonatal deficiency of carnitine palmitoyltransferase II associated with dysgenesis of the brain and kidneys.
Rhabdomyolysis and Cardiomyopathy in a 20-Year-Old Patient with CPT II Deficiency.
Successful orthotopic heart transplantation in CPTII deficiency.
[An unusual case of cardiomyopathy showing carnitine palmitoyltransferase deficiency].
Cardiomyopathy, Dilated
Carnitine palmitoyltransferase in patients with cardiac ischemia due to atherosclerotic coronary artery disease and in patients with idiopathic dilated cardiomyopathy.
Cardiotoxicity
Inhibition of Gene Expression of Carnitine Palmitoyltransferase I and Heart Fatty Acid Binding Protein in Cyclophosphamide and Ifosfamide-Induced Acute Cardiotoxic Rat Models.
Inhibition of mitochondrial carnitine palmitoyltransferases by adriamycin and adriamycin analogues.
Propionyl-L-carnitine as potential protective agent against adriamycin-induced impairment of fatty acid beta-oxidation in isolated heart mitochondria.
Cardiovascular Diseases
Methylation at CPT1A locus is associated with lipoprotein subfraction profiles.
carnitine o-palmitoyltransferase deficiency
"Adult" form of muscular carnitine palmitoyltransferase II deficiency: manifestation in a 2-year-old child.
A case of carnitine palmitoyltransferase II deficiency in human skeletal muscle.
A case of partial carnitine palmitoyltransferase deficiency in a patient undergoing chronic hemodialysis.
A Japanese adult form of CPT II deficiency associated with a homozygous F383Y mutation.
A new nonsense mutation is associated with dysmorphism in lethal neonatal CPTII deficiency.
A newborn case with carnitine palmitoyltransferase II deficiency initially judged as unaffected by acylcarnitine analysis soon after birth.
A novel mutation identified in carnitine palmitoyltransferase II deficiency.
A Novel Mutation in CPT1A Resulting in Hepatic CPT Deficiency.
A novel nonsense mutation (515del4) in muscle carnitine palmitoyltransferase II deficiency.
A novel splice site mutation in neonatal carnitine palmitoyl transferase II deficiency.
A patient with atypical presentation of chronic hepatosteatosis and developmental delay harboring a novel variant in the CPT1A gene.
A retrospective ESI-MS/MS analysis of newborn blood spots from 18 symptomatic patients with organic acid and fatty acid oxidation disorders diagnosed either in infancy or in childhood.
A splice junction mutation in muscle carnitine palmitoyltransferase II deficiency.
A survey of Japanese patients with mitochondrial fatty acid beta-oxidation and related disorders as detected from 1985 to 2000.
A surviving 24-month-old patient with neonatal-onset carnitine palmitoyltransferase II deficiency.
Abbreviated half-lives and impaired fuel utilization in carnitine palmitoyltransferase II variant fibroblasts.
Abnormal CpG island methylation occurs during in vitro differentiation of human embryonic stem cells.
Abnormal oxidative metabolism and O2 transport in muscle phosphofructokinase deficiency.
Acute Muscle Pain in an Adolescent Athlete.
Acute renal failure due to carnitine palmitoyltransferase deficiency.
Acute renal failure due to carnitine palmitoyltransferase II deficiency.
Acute Respiratory Infection Unveiling CPT II Deficiency.
Acute severe encephalopathy related to human herpesvirus-6 infection in a patient with carnitine palmitoyltransferase 2 deficiency carrying thermolabile variants.
Adult carnitine palmitoyltransferase II deficiency: detection of characteristic carnitine esters in serum by tandem mass spectrometry.
An unusual case of placenta increta in a Carnitine palmitoyltransferase deficiency carrier.
Anesthetic management of a parturient with carnitine palmitoyltransferase II deficiency.
Antenatal presentation of carnitine palmitoyltransferase II deficiency.
Approach to generalized weakness and peripheral neuromuscular disease.
Association of CPT II gene with risk of acute encephalitis in Chinese children.
Atypical features of the hepatic form of carnitine palmitoyltransferase deficiency in a Hutterite family.
Atypical Manifestation of CPT1A Deficiency: Hepatosplenomegaly And Nephromegaly.
Biochemical and molecular correlations in carnitine palmitoyltransferase II deficiency.
Biochemical and molecular studies of carnitine palmitoyltransferase II deficiency with hepatocardiomyopathic presentation.
Biochemical evidence for heterozygosity in muscular carnitine palmitoyltransferase deficiency.
Carnitine acyltransferases in normal human skeletal muscle and in muscle of patients with carnitine palmitoyltransferase deficiency.
Carnitine palmitoyl transferase 1A deficiency in an adult with recurrent severe steato hepatitis aggravated by high pathologic or physiologic demands: A roller-coaster for internists.
Carnitine palmitoyl transferase I deficiency presenting as a Reye-like syndrome without hypoglycaemia.
Carnitine palmitoyl transferase II deficiency in an adolescent presenting with rhabdomyolysis and acute renal failure.
Carnitine palmitoyltransferase 1A (CPT1A) P479L prevalence in live newborns in Yukon, Northwest Territories, and Nunavut.
Carnitine palmitoyltransferase 1A deficiency: abnormal muscle biopsy findings in a child presenting with Reye's syndrome.
Carnitine palmitoyltransferase 1A functions to repress FoxO transcription factors to allow cell cycle progression in ovarian cancer.
Carnitine Palmitoyltransferase 1b Deficiency Protects Mice from Diet-Induced Insulin Resistance.
Carnitine Palmitoyltransferase 1b Deficient Mice Develop Severe Insulin Resistance After Prolonged High Fat Diet Feeding.
Carnitine palmitoyltransferase 1C deficiency causes motor impairment and hypoactivity.
Carnitine palmitoyltransferase 2 deficiency: the time-course of blood and urinary acylcarnitine levels during initial L-carnitine supplementation.
Carnitine palmitoyltransferase deficiencies.
Carnitine palmitoyltransferase deficiency in a college athlete: a case report and literature review.
Carnitine palmitoyltransferase deficiency in pregnancy.
Carnitine palmitoyltransferase deficiency.
Carnitine palmitoyltransferase deficiency: a common cause of recurrent myoglobinuria.
Carnitine palmitoyltransferase deficiency: an underdiagnosed condition?
Carnitine palmitoyltransferase I deficiency in neonate identified by dried blood spot free carnitine and acylcarnitine profile.
Carnitine palmitoyltransferase IA polymorphism P479L is common in Greenland Inuit and is associated with elevated plasma apolipoprotein A-I.
Carnitine palmitoyltransferase II (CPT II) deficiency: genotype-phenotype analysis of 50 patients.
Carnitine Palmitoyltransferase II Deficiency (CPT II) Followed By Rhabdomyolysis and Acute Kidney Injury.
Carnitine palmitoyltransferase II deficiency and post-COVID vaccination rhabdomyolysis.
Carnitine palmitoyltransferase II deficiency due to a novel gene variant in a patient with rhabdomyolysis and ARF.
Carnitine palmitoyltransferase II deficiency in a prenatal case with polycystic kidney disease-like phenotype.
Carnitine palmitoyltransferase II deficiency with a focus on newborn screening.
Carnitine palmitoyltransferase II deficiency with normal carnitine palmitoyltransferase I in skeletal muscle and leucocytes.
Carnitine palmitoyltransferase II deficiency: a clinical, biochemical, and molecular review.
Carnitine palmitoyltransferase II deficiency: a new cause of recurrent pancreatitis.
Carnitine palmitoyltransferase II deficiency: diagnosis by molecular analysis of blood.
Carnitine palmitoyltransferase II deficiency: molecular and biochemical analysis of 32 patients.
Carnitine palmitoyltransferase II deficiency: structure of the gene and characterization of two novel disease-causing mutations.
Carnitine palmitoyltransferase II deficiency: successful anaplerotic diet therapy.
Carnitine Palmitoyltransferase-1b (CPT1b) Deficiency Aggravates Pressure-Overload-Induced Cardiac Hypertrophy due to Lipotoxicity.
Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects.
Carrier Frequency of a Common Mutation of Carnitine Palmitoyltransferase 1A Deficiency and Long-Term Follow-Up in Finland.
Cause of recurrent rhabdomyolysis, carnitine palmitoyltransferase II deficiency and novel pathogenic mutation.
Central nervous system disorders and possible brain type carnitine palmitoyltransferase II deficiency.
Ceramide levels regulated by carnitine palmitoyltransferase 1C control dendritic spine maturation and cognition.
Cerebral Lipid Accumulation Detected by MRS in a Child with Carnitine Palmitoyltransferase 2 Deficiency: A Case Report and Review of the Literature on Genetic Etiologies of Lipid Peaks on MRS.
Characterisation of carnitine palmitoyltransferases in patients with a carnitine palmitoyltransferase deficiency: implications for diagnosis and therapy.
Cholestatic Jaundice Associated with Carnitine Palmitoyltransferase IA Deficiency.
Clinical and biochemical heterogeneity in an Italian family with CPT II deficiency due to Ser 113 Leu mutation.
Clinical and genetic characteristics of patients with fatty acid oxidation disorders identified by newborn screening.
Clinical varieties of carnitine and carnitine palmitoyltransferase deficiency.
Clinically symptomatic heterozygous carnitine palmitoyltransferase II (CPT II) deficiency.
Complementation analysis of carnitine palmitoyltransferase I and II defects.
CPT2 gene mutations resulting in lethal neonatal or severe infantile carnitine palmitoyltransferase II deficiency.
Defects in activation and transport of fatty acids.
Detection of Early Onset Carnitine Palmitoyltransferase II Deficiency by Newborn Screening: Should CPT II Deficiency Be a Primary Disease Target?
Detection of neonatal carnitine palmitoyltransferase II deficiency by expanded newborn screening with tandem mass spectrometry.
Diagnostic assessment and long-term follow-up of 13 patients with Very Long-Chain Acyl-Coenzyme A dehydrogenase (VLCAD) deficiency.
Diagnostic pitfall in antenatal manifestations of CPT II deficiency.
Different ketogenic response to medium-chain triglycerides and to long-chain triglycerides in a case of muscular carnitine palmitoyltransferase deficiency.
Different sensitivities of CPT I and CPT II for inhibition by l-aminocarnitine in human skeletal muscle.
Disorders of fatty acid oxidation and autosomal recessive polycystic kidney disease-different clinical entities and comparable perinatal renal abnormalities.
Effect of diet on exercise tolerance in carnitine palmitoyltransferase II deficiency.
Elevated plasma carnitine in the hepatic form of carnitine palmitoyltransferase-1 deficiency.
Evaluation of Metabolic Defects in Fatty Acid Oxidation Using Peripheral Blood Mononuclear Cells Loaded with Deuterium-Labeled Fatty Acids.
Exercise tolerance in carnitine palmitoyltransferase II deficiency with IV and oral glucose.
Exercise, Nutrition, and Supplements in the Muscle Carnitine Palmitoyl-Transferase II Deficiency: New Theoretical Bases for Potential Applications.
Exertional rhabdomyolysis leading to acute kidney injury: when genetic defects are diagnosed in adult life.
Exome-Based Rare-Variant Analyses in CKD.
Expression analysis of two mutations in carnitine palmitoyltransferase IA deficiency.
Fatal carnitine palmitoyltransferase II deficiency in a newborn: new phenotypic features.
Fatty Acid oxidation disorders in a chinese population in taiwan.
Features of carnitine palmitoyltransferase type I deficiency.
First Japanese Case of Carnitine Palmitoyltransferase II Deficiency with the Homozygous Point Mutation S113L.
Fuel utilization in subjects with carnitine palmitoyltransferase 2 gene mutations.
Functional analysis of iPSC-derived myocytes from a patient with carnitine palmitoyltransferase II deficiency.
Functional and structural basis of carnitine palmitoyltransferase 1A deficiency.
Genetic risk factors associated with lipid-lowering drug-induced myopathies.
Genetics of carnitine palmitoyltransferase II deficiencies.
Genotype-phenotype correlations in a large series of patients with muscle type CPT II deficiency.
Hepatic carnitine palmitoyltransferase I deficiency presenting as maternal illness in pregnancy.
Hepatic carnitine palmitoyltransferase I deficiency: acylcarnitine profiles in blood spots are highly specific.
High frequency of ETFDH c.250G>A mutation in Taiwanese patients with late-onset lipid storage myopathy.
Human liver mitochondrial carnitine palmitoyltransferase I: characterization of its cDNA and chromosomal localization and partial analysis of the gene.
Hypercholesterolemia treatment in a patient with family hypercholesterolemia and myopathy due to carnitine palmitoyltransferase II deficiency with PCSK9 inhibitors.
Hyperlipidaemia due to carnitine palmitoyltransferase I deficiency.
Identification by mutagenesis of a conserved glutamate (Glu487) residue important for catalytic activity in rat liver carnitine palmitoyltransferase II.
Identification of 16 new disease-causing mutations in the CPT2 gene resulting in carnitine palmitoyltransferase II deficiency.
Identification of a common mutation in the carnitine palmitoyltransferase II gene in familial recurrent myoglobinuria patients.
Identification of four novel mutations in patients with carnitine palmitoyltransferase II (CPT II) deficiency.
Identification of novel mutations in Spanish patients with muscle carnitine palmitoyltransferase II deficiency.
Immunoquantitative analysis of human carnitine palmitoyltransferase I and II defects.
In vivo stable isotope studies in three patients affected with mitochondrial fatty acid oxidation disorders: limited diagnostic use of 1-13C fatty acid breath test using bolus technique.
Increased serum lactate dehydrogenase isoenzyme 1 and "flipped" LD-1/LD-2 ratio in myopathy associated with partial carnitine palmitoyltransferase deficiency.
Infantile form of carnitine palmitoyltransferase II deficiency in a girl with rapid fatal onset.
Infantile form of carnitine palmitoyltransferase II deficiency with hepatomuscular symptoms and sudden death. Physiopathological approach to carnitine palmitoyltransferase II deficiencies.
Inheritance of the S113L mutation within an inbred family with carnitine palmitoyltransferase enzyme deficiency.
Inhibition of carnitine palmitoyltransferase in normal human skeletal muscle and in muscle of patients with carnitine palmitoyltransferase deficiency by long- and short-chain acylcarnitine and acyl-coenzyme A.
Laryngeal spasm mimicking asthma and vitamin d deficiency.
Lethal neonatal and severe late infantile forms of carnitine palmitoyltransferase II deficiency associated with compound heterozygosity for different protein truncation mutations.
Lethal neonatal carnitine palmitoyltransferase II deficiency: an unusual presentation of a rare disorder.
Lethal neonatal presentation of carnitine palmitoyltransferase I deficiency.
Limited trypsin proteolysis renders carnitine palmitoyltransferase insensitive to inhibition by malonyl-CoA in patients with muscle carnitine palmitoyltransferase deficiency.
Lipids detected by brain MRS during coma caused by carnitine palmitoyltransferase 1 deficiency.
Long-chain acyl-CoA profiles in cultured fibroblasts from patients with defects in fatty acid oxidation.
Long-term follow-up of bezafibrate treatment in patients with the myopathic form of carnitine palmitoyltransferase 2 deficiency.
Macro creatine kinase in a case of carnitine palmitoyltransferase deficiency.
Malignant hyperthermia-like syndrome and carnitine palmitoyltransferase II deficiency with heterozygous R503C mutation.
Malonyl-CoA abnormal inhibition of residual enzyme activity in carnitine palmitoyltransferase deficiency.
Metabolic autopsy with next generation sequencing in sudden unexpected death in infancy: Postmortem diagnosis of fatty acid oxidation disorders.
Metabolic characterization of a woman homozygous for the Ser113Leu missense mutation in carnitine palmitoyl transferase II.
Molecular analysis in Spanish patients with muscle carnitine palmitoyltransferase deficiency.
Molecular analysis of carnitine palmitoyltransferase II deficiency with hepatocardiomuscular expression.
Molecular and enzymatic characterization of a unique carnitine palmitoyltransferase 1A mutation in the Hutterite community.
Molecular assay for detection of the common carnitine palmitoyltransferase 1A 1436(C>T) mutation.
Molecular basis of hepatic carnitine palmitoyltransferase I deficiency.
Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.
Muscle Carnitine Palmitoyltransferase II (CPT II) Deficiency: A Conceptual Approach.
Muscle Carnitine Palmitoyltransferase II Deficiency: A Review of Enzymatic Controversy and Clinical Features.
Muscle carnitine palmitoyltransferase II deficiency: clinical and molecular genetic features and diagnostic aspects.
Muscular carnitine palmitoyltransferase II deficiency in infancy.
Mutations of carnitine palmitoyltransferase II (CPT II) in Japanese patients with CPT II deficiency.
Myocardial carnitine and carnitine palmitoyltransferase deficiencies in patients with severe heart failure.
Myopathies due to enzyme deficiencies.
Need for strict clinical management of patients with carnitine palmitoyltransferase II deficiency: Experience with two cases detected by expanded newborn screening.
Neonatal carnitine palmitoyltransferase deficiency: a case with a muscular presentation.
Neonatal carnitine palmitoyltransferase II deficiency associated with Dandy-Walker syndrome and sudden death.
Neonatal Carnitine Palmitoyltransferase II Deficiency: A Lethal Entity.
Neonatal carnitine palmitoyltransferase II deficiency: failure of treatment despite prolonged survival.
Neonatal metabolic myopathies.
New developments in the diagnosis and investigation of mitochondrial fatty acid oxidation disorders.
Newborn screening for carnitine palmitoyltransferase II deficiency using (C16+C18:1)/C2: Evaluation of additional indices for adequate sensitivity and lower false-positivity.
No carnitine palmitoyltransferase deficiency in skeletal muscle in 18 malignant hyperthermia susceptible individuals.
Noninvasive evaluation of adult onset myopathy from carnitine palmitoyl transferase II deficiency using proton magnetic resonance spectroscopy.
Normal FGF-21-Serum Levels in Patients with Carnitine Palmitoyltransferase II (CPT II) Deficiency.
Normal Levels of Plasma Free Carnitine and Acylcarnitines in Follow-Up Samples from a Presymptomatic Case of Carnitine Palmitoyl Transferase 1 (CPT1) Deficiency Detected Through Newborn Screening in Denmark.
Normal protein content but abnormally inhibited enzyme activity in muscle carnitine palmitoyltransferase II deficiency.
Novel metabolic and molecular findings in hepatic carnitine palmitoyltransferase I deficiency.
Novel mutation in carnitine palmitoyltransferase 1A detected through newborn screening for a presymptomatic case in China: a case report.
Novel mutation in the CPT II gene in a child with periodic febrile myalgia and myoglobinuria.
Novel mutations associated with carnitine palmitoyltransferase II deficiency.
Novel mutations in CPT 1A define molecular heterogeneity of hepatic carnitine palmitoyltransferase I deficiency.
Novel mutations in myopathic form of carnitine palmitoyltransferase II deficiency in a Chinese patient.
Novel Mutations in the CPT1A Gene Identified in the Patient Presenting Jaundice as the First Manifestation of Carnitine Palmitoyltransferase 1A Deficiency.
Novel mutations in the gene encoding very long-chain acyl-CoA dehydrogenase identified in patients with partial carnitine palmitoyltransferase II deficiency.
Novel Regulation of the Synthesis of ?-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic Acid (AMPA) Receptor Subunit GluA1 by Carnitine Palmitoyltransferase 1C (CPT1C) in the Hippocampus.
Nutrition and Exercise in a Case of Carnitine Palmitoyl-Transferase II Deficiency.
Partial muscle carnitine palmitoyltransferase-A deficiency. Rhabdomyolysis associated with transiently decreased muscle carnitine content after ibuprofen therapy.
Pathophysiological approach to carnitine palmitoyltransferase II deficiencies.
Phenotype of carnitine palmitoyltransferase II (CPT II) deficiency: A questionnaire-based survey.
Phenotypic variability among first-degree relatives with carnitine palmitoyltransferase II deficiency.
Post-mortem MRI reveals CPT2 deficiency after sudden infant death.
Postinfectious Rhabdomyolysis in a 5-Year-Old Boy: When to Look a Little Deeper.
Postoperative coma in a child with carnitine palmitoyltransferase I deficiency.
Prenatal diagnosis of carnitine palmitoyltransferase 2 deficiency in chorionic villi: a novel approach.
Prevalence and Distribution of the c.1436C?T Sequence Variant of Carnitine Palmitoyltransferase 1A among Alaska Native Infants.
Profound carnitine palmitoyltransferase II deficiency.
Quantitative acylcarnitine profiling in fibroblasts using [U-13C] palmitic acid: an improved tool for the diagnosis of fatty acid oxidation defects.
Rapid, cost-effective gene mutation screening for carnitine palmitoyltransferase II deficiency using whole blood on filter paper.
Rate-dependent distal renal tubular acidosis and carnitine palmitoyltransferase I deficiency.
Rationale for a conditional knockout mouse model to study carnitine palmitoyltransferase I deficiencies.
Recurrent metabolic decompensation in profound carnitine palmitoyltransferase II deficiency.
Recurrent Myalgia since Early Infancy-Misleading Clinical Course in a Child with Carnitine Palmitoyltransferase-II Deficiency.
Recurrent myoglobinuria due to carnitine palmitoyltransferase II deficiency: clinical, biochemical, and genetic features of adult-onset cases.
Recurrent myoglobinuria due to carnitine palmitoyltransferase II deficiency: expression of the molecular phenotype in cultured muscle cells.
Recurrent rhabdomyolysis caused by carnitine palmitoyltransferase II deficiency, common but under-recognised: Lessons to be learnt.
Recurrent rhabdomyolysis in a collegiate athlete: a case report.
Repeat MRI in acute rhabdomyolysis: correlation with clinicopathological findings.
Repeated and progressive rhabdomyolysis due to a novel carnitine palmitoyltransferase II gene variant in an adult male: A case report.
Retrospective review of Japanese sudden unexpected death in infancy: The importance of metabolic autopsy and expanded newborn screening.
Reversible asphyxial status in a newborn due to neonatal form of carnitine palmitoyltransferase II deficiency.
Rhabdomyolysis and Cardiomyopathy in a 20-Year-Old Patient with CPT II Deficiency.
Rhabdomyolysis and respiratory failure: rare presentation of carnitine palmityl-transferase II deficiency.
Rhabdomyolysis caused by carnitine palmitoyltransferase 2 deficiency: A case report and systematic review of the literature.
Rhabdomyolysis with different etiologies in childhood.
Screening for carnitine palmitoyltransferase II deficiency by tandem mass spectrometry.
Secondary carnitine palmitoyltransferase deficiency in chronic renal failure and secondary hyperparathyroidism.
Selective carnitine palmitoyltransferase deficiency in fibroblasts from a patient with muscle CPT deficiency.
Selective screening for fatty acid oxidation disorders by tandem mass spectrometry: difficulties in practical discrimination.
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Serious pregnancy complications in a patient with previously undiagnosed carnitine palmitoyltransferase 1 deficiency.
Severe Hyperammonemic Encephalopathy Requiring Dialysis Aggravated by Prolonged Fasting and Intermittent High Fat Load in a Ramadan Fasting Month in a Patient with CPTII Homozygous Mutation.
SEVERE INFANTILE CARNITINE PALMITOYLTRANSFERASE II (CPT II) DEFICIENCY IN 19-WEEK FETAL SIBS.
Severe periodic febrile myalgia in infancy due to carnitine palmitoyltransferase deficiency.
Severe rhabdomyolysis in homozygote carnitine palmitoyltransferase II deficiency.
Severe rhabdomyolysis with hypoglycemia in an adult patient with carnitine palmitoyltransferase II deficiency.
Skeletal muscle-specific CPT1 deficiency elevates lipotoxic intermediates but preserves insulin sensitivity.
Stabilization of the thermolabile variant S113L of carnitine palmitoyltransferase II.
Statin use in carnitine palmitoyltransferase II deficiency.
Study of Carnitine/Acylcarnitine and Aminoacid Profile in children and adults with Acute Liver Failure.
Subclinical effects of long-chain fatty acid ?-oxidation deficiency on the adult heart: A case-control magnetic resonance study.
Successful long-term treatment of hepatic carnitine palmitoyltransferase I deficiency and a novel mutation.
Successful orthotopic heart transplantation in CPTII deficiency.
Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program.
Task force guidelines handbook: EFNS guidelines on diagnosis and management of fatty acid mitochondrial disorders.
The crystal structure of carnitine palmitoyltransferase 2 and implications for diabetes treatment.
The diagnosis of carnitine palmitoyltransferase II deficiency is now possible in small skeletal muscle biopsies.
The role of mitochondria in statin-induced myopathy.
Therapeutic advances in the management of Pompe disease and other metabolic myopathies.
Trifunctional enzyme deficiency: adult presentation of a usually fatal beta-oxidation defect.
Two CPT2 mutations in three Japanese patients with carnitine palmitoyltransferase II deficiency: functional analysis and association with polymorphic haplotypes and two clinical phenotypes.
Two novel gene mutations (Glu174-->Lys, Phe383-->Tyr) causing the "hepatic" form of carnitine palmitoyltransferase II deficiency.
Unmasking of carnitine palmitoyltransferase deficiency during an acute exacerbation of asthma complicated by rhabdomyolysis in a soldier.
Unrecognized High Occurrence of Genetically Confirmed Hereditary Carnitine Palmitoyltransferase II Deficiency in an Austrian Family Points to the Ongoing Underdiagnosis of the Disease.
Utility of Genetic Testing for Confirmation of Abnormal Newborn Screening in Disorders of Long-Chain Fatty Acids: A Missed Case of Carnitine Palmitoyltransferase 1A (CPT1A) Deficiency.
[A case report of carnitine palmitoyltransferase II deficiency]
[A female case of carnitine palmitoyltransferase deficiency]
[Acute respiratory insufficiency and carnitine palmitoyltransferase deficiency]
[Alterations of mitochondria in metabolic diseases. Carnitine deficiency, carnitine palmitoyltransferase deficiency and beta oxidation.]
[An unusual case of cardiomyopathy showing carnitine palmitoyltransferase deficiency].
[Analysis of a child with carnitine palmitoyl transferase 1A deficiency due to variant of CPT1A gene].
[Anesthetic management of a patient with carnitine palmitoyltransferase deficiency with a history of rhabdomyolysis].
[Application of tandem mass spectrometry on the diagnosis of fatty acid oxidation disorders]
[Carnitine palmitoyltransferase deficiency (I, II)]
[Carnitine palmitoyltransferase deficiency as the cause of effort rhabdomyolysis]
[Carnitine palmitoyltransferase deficiency in a patient with severe psychomotor retardation]
[Carnitine palmitoyltransferase deficiency: fatty acid utilization defect causing myopathy]
[Carnitine palmitoyltransferase deficiency]
[Carnitine palmitoyltransferase I deficiency]
[Carnitine palmitoyltransferase II deficiency]
[Clinical aspects of disorders of lipid metabolism in humans. II. Carnitine palmitoyltransferase deficiency]
[Clinical features and gene mutations of 6 patients with carnitine palmitoyltransferase 1A deficiency].
[Clinical features and genetic analysis of a case with carnitine palmitoyltransferase 1A deficiency].
[CPT2 gene mutation analysis and prenatal diagnosis in a family with carnitine palmitoyltransferase II deficiency].
[Exercise-intolerance and exercise-induced rhabdomyolisis: etiology and diagnosis]
[Exploration of exercise intolerance by 31P NMR spectroscopy of calf muscles coupled with MRI and ergometry]
[Heterogeneity of carnitine palmitoyltransferase deficiencies. Deficiency of CPT I in the hepatic form and CPT II in the muscular form]
[Identification of missense mutations and haplotyping of carnitine palmitoyltransferase II gene]
[Intolerance to exercise caused by carnitine palmitoyltransferase deficiency]
[Lipid storage myopathies--carnitine deficiency, carnitine palmitoyltransferase deficiency, pyruvate decarboxylase deficiency]
[Metabolic intolerance to exercise]
[Molecular analysis of a patient with carnitine palmitoyltransferase II deficiency]
[Myoglobinuria and carnitine palmitoyltransferase deficiency. Diagnostic procedure and differential diagnosis]
[Myopathy due to carnitine palmitoyltransferase deficiency. Report of 2 cases with enzymatic analyses on muscle tissue]
[Myopathy in the course of carnitine palmitoyltransferase II deficiency].
[New approaches for the treatment of metabolic myopathies]
[Non-ketotic hypoglycemia caused by carnitine palmitoyl transferase 1 deficiency]
[Recurrent myoglobinuria in a girl due to carnitine palmitoyltransferase deficiency]
[Rhabdomyolysis caused by carnitine palmitoyltransferase deficiency. Prevention of acute kidney failure]
[Rhabdomyolysis caused by carnitine palmitoyltransferase deficiency]
[Rhabdomyolysis due to muscle enzyme deficiencies]
[Rhabdomyolysis in carnitine palmitoyltransferase deficiency]
[Rhabdomyolysis in carnitine palmitoyltransferase II deficiency: developments in pathophysiology, diagnosis and therapy]
[Tandem mass spectrometry analysis and genetic diagnosis of neonates with fatty acid oxidation disorders in central and northern regions of Guangxi].
[Type II carnitine palmitoyl transferase deficiency complicated by acute respiratory failure]
Cataplexy
Polymorphism located between CPT1B and CHKB, and HLA-DRB1*1501-DQB1*0602 haplotype confer susceptibility to CNS hypersomnias (essential hypersomnia).
Central Nervous System Diseases
Central nervous system disorders and possible brain type carnitine palmitoyltransferase II deficiency.
Dysregulation of metabolic pathways by carnitine palmitoyl-transferase 1 plays a key role in central nervous system disorders: experimental evidence based on animal models.
Cholestasis
Inhibition of carnitine acyltransferase activities by bile acids in rat liver peroxisomes.
Colonic Neoplasms
Dysregulated Free Fatty Acid Receptor 2 Exacerbates Colonic Adenoma Formation in Apc Min/+ Mice: Relation to Metabolism and Gut Microbiota Composition.
Enhancing cancer-associated fibroblast fatty acid catabolism within a metabolically challenging tumor microenvironment drives colon cancer peritoneal metastasis.
iRGD-modified exosomes effectively deliver CPT1A siRNA to colon cancer cells, reversing oxaliplatin resistance by regulating fatty acid oxidation.
Upregulation of CPT1A is essential for the tumor-promoting effect of adipocytes in colon cancer.
Colorectal Neoplasms
Cellular Metabolism and Dose Reveal Carnitine-Dependent and -Independent Mechanisms of Butyrate Oxidation in Colorectal Cancer Cells.
Enhancing cancer-associated fibroblast fatty acid catabolism within a metabolically challenging tumor microenvironment drives colon cancer peritoneal metastasis.
Coma
Features of carnitine palmitoyltransferase type I deficiency.
Lipids detected by brain MRS during coma caused by carnitine palmitoyltransferase 1 deficiency.
Postoperative coma in a child with carnitine palmitoyltransferase I deficiency.
Communicable Diseases
The p.P479L variant in CPT1A is associated with infectious disease in a BC First Nation.
Compartment Syndromes
Repeat MRI in acute rhabdomyolysis: correlation with clinicopathological findings.
Confusion
Egocentric disorientation and heading disorientation: evaluation by a new test named card placing test.
Coronary Artery Disease
Carnitine palmitoyltransferase in patients with cardiac ischemia due to atherosclerotic coronary artery disease and in patients with idiopathic dilated cardiomyopathy.
COVID-19
Carnitine palmitoyltransferase II deficiency and post-COVID vaccination rhabdomyolysis.
Mitochondrial induced T cell apoptosis and aberrant myeloid metabolic programs define distinct immune cell subsets during acute and recovered SARS-CoV-2 infection.
Cytochrome-c Oxidase Deficiency
Neonatal metabolic myopathies.
Dandy-Walker Syndrome
Neonatal carnitine palmitoyltransferase II deficiency associated with Dandy-Walker syndrome and sudden death.
Deficiency Diseases
Identification by mutagenesis of a conserved glutamate (Glu487) residue important for catalytic activity in rat liver carnitine palmitoyltransferase II.
Demyelinating Diseases
Inhibition of fatty acid metabolism ameliorates disease activity in an animal model of multiple sclerosis.
Diabetes Mellitus
Alterations of heart function and Na+-K+-ATPase activity by etomoxir in diabetic rats.
Carnitine palmitoyltransferase 2: analysis of membrane association and complex structure with a substrate analog.
Lack of association of CPT1A polymorphisms or haplotypes on hepatic lipid content or insulin resistance in Japanese individuals with type 2 diabetes mellitus.
MiRNA-324-5p inhibits inflammatory response of diabetic vessels by targeting CPT1A.
Mitochondrial regulators of fatty acid metabolism reflect metabolic dysfunction in type 2 diabetes mellitus.
Possible new therapeutic approach in diabetes mellitus by inhibition of carnitine palmitoyltransferase 1 (CPT1).
The crystal structure of carnitine palmitoyltransferase 2 and implications for diabetes treatment.
Diabetes Mellitus, Type 2
AMPK in the small intestine in normal and pathophysiological conditions.
Aqueous-Methanol Extracts of Orange-Fleshed Sweet Potato (Ipomoeabatatas) Ameliorate Oxidative Stress and Modulate Type 2 Diabetes Associated Genes in Insulin Resistant C2C12 Cells.
C75 [4-methylene-2-octyl-5-oxo-tetrahydro-furan-3-carboxylic acid] activates carnitine palmitoyltransferase-1 in isolated mitochondria and intact cells without displacement of bound malonyl CoA.
Carnitine palmitoyltransferase 2: analysis of membrane association and complex structure with a substrate analog.
Enhanced fatty acid oxidation in adipocytes and macrophages reduces lipid-induced triglyceride accumulation and inflammation.
Fatty Acid Metabolites Combine with Reduced ? Oxidation to Activate Th17 Inflammation in Human Type 2 Diabetes.
Inhibition by etomoxir of carnitine palmitoyltransferase I reduces hepatic glucose production and plasma lipids in non-insulin-dependent diabetes mellitus.
Lack of association of CPT1A polymorphisms or haplotypes on hepatic lipid content or insulin resistance in Japanese individuals with type 2 diabetes mellitus.
MiRNA-324-5p inhibits inflammatory response of diabetic vessels by targeting CPT1A.
Mitochondrial regulators of fatty acid metabolism reflect metabolic dysfunction in type 2 diabetes mellitus.
Diabetes, Gestational
Association of CPT1A gene polymorphism with the risk of gestational diabetes mellitus: a case-control study.
Diabetic Cardiomyopathies
[Screening and analysis of early cardiopathology-related gene in type 2 diabetes mellitus]
Diabetic Ketoacidosis
Hepatic mitochondrial function in ketogenic states. Diabetes, starvation, and after growth hormone administration.
Diabetic Nephropathies
Mitochondrial carnitine palmitoyltransferase 2 is involved in N?-(carboxymethyl)-lysine-mediated diabetic nephropathy.
Disorders of Excessive Somnolence
Polymorphism located between CPT1B and CHKB, and HLA-DRB1*1501-DQB1*0602 haplotype confer susceptibility to CNS hypersomnias (essential hypersomnia).
Dyslipidemias
AMPK in the small intestine in normal and pathophysiological conditions.
Effect of pigeon pea (Cajanus cajan L.) on high-fat diet-induced hypercholesterolemia in hamsters.
Preventive effect of small-leaved Kuding tea (Ligustrum robustum) on high-diet-induced obesity in C57BL/6J mice.
Encephalitis
Association of CPT II gene with risk of acute encephalitis in Chinese children.
Association of the Polymorphism of rs1799822 on Carnitine Palmitoyltransferase II Gene with Severe Enterovirus 71 Encephalitis in Chinese Children.
Pharmacological inhibition of carnitine palmitoyl transferase 1 inhibits and reverses experimental autoimmune encephalitis in rodents.
Encephalomyelitis
CPT1A plays a key role in the development and treatment of multiple sclerosis and experimental autoimmune encephalomyelitis.
Encephalomyelitis, Autoimmune, Experimental
CPT1A plays a key role in the development and treatment of multiple sclerosis and experimental autoimmune encephalomyelitis.
Endotoxemia
Differential effects of neonatal endotoxemia on heart and kidney carnitine palmitoyl transferase I.
Epilepsy
Association of CYP2C9, CYP2A6, ACSM2A, and CPT1A gene polymorphisms with adverse effects of valproic acid in Chinese patients with epilepsy.
[Analysis of a child with carnitine palmitoyl transferase 1A deficiency due to variant of CPT1A gene].
Epstein-Barr Virus Infections
Repeat MRI in acute rhabdomyolysis: correlation with clinicopathological findings.
Fatigue Syndrome, Chronic
Long-chain acylcarnitine deficiency in patients with chronic fatigue syndrome. Potential involvement of altered carnitine palmitoyltransferase-I activity.
Fatty Liver
A common haplotype of carnitine palmitoyltransferase 1b is associated with the metabolic syndrome.
A patient with atypical presentation of chronic hepatosteatosis and developmental delay harboring a novel variant in the CPT1A gene.
Adaptations of hepatic lipid metabolism and mitochondria in dairy cows with mild fatty liver.
Altered expression of carnitine palmitoyltransferase II in liver, muscle, and heart of mouse strain with juvenile visceral steatosis.
Ananas comosus L. Leaf Phenols and p-Coumaric Acid Regulate Liver Fat Metabolism by Upregulating CPT-1 Expression.
Are miRNA-103, miRNA-107 and miRNA-122 Involved in the Prevention of Liver Steatosis Induced by Resveratrol?
Changes in hepatic lipogenic and oxidative enzymes and glucose homeostasis induced by an acetyl-l-carnitine and nicotinamide treatment in dyslipidaemic insulin-resistant rats.
Chinese medicine formula lingguizhugan decoction improves Beta-oxidation and metabolism of Fatty Acid in high-fat-diet-induced rat model of Fatty liver disease.
Chronic administration of recombinant IL-6 upregulates lipogenic enzyme expression and aggravates high-fat-diet-induced steatosis in IL-6-deficient mice.
Cyclooxygenase inhibition upregulates liver carnitine palmitoyltransferase 1A (CPT1A) expression and improves fatty liver.
Effect of methotrexate on long-chain fatty acid metabolism in liver of rats fed a standard or a defined, choline-deficient diet.
Effects of post-suckling n-3 polyunsaturated fatty acids: prevention of dyslipidemia and liver steatosis induced in rats by a sucrose-rich diet during pre- and post-natal life.
Increased expression of carnitine palmitoyltransferase I gene is repressed by administering L-carnitine in the hearts of carnitine-deficient juvenile visceral steatosis mice.
Inhibition of Carnitine Palmitoyltransferase 1A Aggravates Fatty Liver Graft Injury via Promoting Mitochondrial Permeability Transition.
Inhibitory effect of Ginkgo biloba extract on fatty liver: Regulation of carnitine palmitoyltransferase 1a and fatty acid metabolism.
Involvement of adenosine monophosphate-activated protein kinase in the influence of timed high-fat evening diet on the hepatic clock and lipogenic gene expression in mice.
Lack of association of CPT1A polymorphisms or haplotypes on hepatic lipid content or insulin resistance in Japanese individuals with type 2 diabetes mellitus.
Liver-specific deletion of IGF2 mRNA binding protein-2/IMP2 reduces hepatic fatty acid oxidation and increases hepatic triglyceride accumulation.
Low abundance of mitofusin 2 in dairy cows with moderate fatty liver is associated with alterations in hepatic lipid metabolism.
Platycodon grandiflorum-derived saponin enhances exercise function, skeletal muscle protein synthesis, and mitochondrial function.
Preliminary studies on hepatic carnitine palmitoyltransferase in dairy cattle with or without fatty liver.
Follicular Cyst
Alterations in key metabolic sensors involved in bovine cystic ovarian disease.
Gastroenteritis
Association of the CPT1A p.P479L Metabolic Gene Variant With Childhood Respiratory and Other Infectious Illness in Nunavut.
Gastrointestinal Neoplasms
Inhibition of fatty acid catabolism augments the efficacy of oxaliplatin-based chemotherapy in gastrointestinal cancers.
Genetic Diseases, Inborn
Defects in activation and transport of fatty acids.
Glioblastoma
High grade glioblastoma is associated with aberrant expression of ZFP57, a protein involved in gene imprinting, and of CPT1A and CPT1C that regulate fatty acid metabolism.
Inhibition of fatty acid oxidation by etomoxir impairs NADPH production and increases reactive oxygen species resulting in ATP depletion and cell death in human glioblastoma cells.
Very long chain fatty acid ?-oxidation in astrocytes: contribution of the ABCD1-dependent and -independent pathways.
Glioma
Elevated expression of fatty acid synthase and nuclear localization of carnitine palmitoyltransferase 1C are common among human gliomas.
Impact of mitochondrial beta-oxidation in fatty acid-mediated inhibition of glioma cell proliferation.
Inhibition of Metabolic Shift can Decrease Therapy Resistance in Human High-Grade Glioma Cells.
Poly-ion complex micelles effectively deliver CoA-conjugated CPT1A inhibitors to modulate lipid metabolism in brain cells.
Glucose Intolerance
Enhanced susceptibility of Cpt1c knockout mice to glucose intolerance induced by a high-fat diet involves elevated hepatic gluconeogenesis and decreased skeletal muscle glucose uptake.
Glucometabolic consequences of acute and prolonged inhibition of fatty acid oxidation.
glycogen phosphorylase deficiency
Recurrent rhabdomyolysis in a collegiate athlete: a case report.
[Rhabdomyolysis due to muscle enzyme deficiencies]
Glycogen Storage Disease
Glycerolphosphate acyltransferase, dihydroxyacetonephosphate acyltransferase and carnitine palmitoyltransferase in a glycogen storage disease (gsd/gsd) rat.
Metabolic myopathies.
Neonatal metabolic myopathies.
Therapeutic advances in the management of Pompe disease and other metabolic myopathies.
Glycogen Storage Disease Type II
Therapeutic advances in the management of Pompe disease and other metabolic myopathies.
Glycogen Storage Disease Type V
Carnitine palmitoyltransferase deficiency in a college athlete: a case report and literature review.
Genetic risk factors associated with lipid-lowering drug-induced myopathies.
Recurrent rhabdomyolysis in a collegiate athlete: a case report.
The role of mitochondria in statin-induced myopathy.
Therapeutic options in other metabolic myopathies.
[Rhabdomyolysis due to muscle enzyme deficiencies]
Glycogen Storage Disease Type VII
Abnormal oxidative metabolism and O2 transport in muscle phosphofructokinase deficiency.
Heart Diseases
CPT1A: the future of heart disease detection and personalized medicine?
Heart Failure
Carnitine palmitoyl transferase-I inhibition prevents ventricular remodeling and delays decompensation in pacing-induced heart failure.
Carnitine Palmitoyltransferase-1b (CPT1b) Deficiency Aggravates Pressure-Overload-Induced Cardiac Hypertrophy due to Lipotoxicity.
Carnitine palmitoyltransferase-I, a new target for the treatment of heart failure: perspectives on a shift in myocardial metabolism as a therapeutic intervention.
Effect of Allopurinol on Myocardial Energy Metabolism in Chronic Heart Failure Rats After Myocardial Infarct.
Improving characterization of hypertrophy-induced murine cardiac dysfunction using four-dimensional ultrasound-derived strain mapping.
Metoprolol represses PGC1alpha-mediated carnitine palmitoyltransferase-1B expression in the diabetic heart.
Myocardial carnitine and carnitine palmitoyltransferase deficiencies in patients with severe heart failure.
Sarcoplasmic reticulum function and carnitine palmitoyltransferase-1 inhibition during progression of heart failure.
The metabolic modulators, Etomoxir and NVP-LAB121, fail to reverse pressure overload induced heart failure in vivo.
Hematologic Diseases
Inhibition of CPT1a as a prognostic marker can synergistically enhance the antileukemic activity of ABT199.
Hematologic Neoplasms
Targeting the leukemia cell metabolism by the CPT1a inhibition: functional preclinical effects in leukemias.
Hepatic Encephalopathy
A patient with atypical presentation of chronic hepatosteatosis and developmental delay harboring a novel variant in the CPT1A gene.
Carnitine palmitoyltransferase 1A (CPT1A) P479L prevalence in live newborns in Yukon, Northwest Territories, and Nunavut.
Novel mutation in carnitine palmitoyltransferase 1A detected through newborn screening for a presymptomatic case in China: a case report.
Novel Mutations in the CPT1A Gene Identified in the Patient Presenting Jaundice as the First Manifestation of Carnitine Palmitoyltransferase 1A Deficiency.
Postoperative coma in a child with carnitine palmitoyltransferase I deficiency.
Hepatitis
Carnitine palmitoyl transferase 1A deficiency in an adult with recurrent severe steato hepatitis aggravated by high pathologic or physiologic demands: A roller-coaster for internists.
Hepatitis C
Hepatitis C virus infection down-regulates the expression of peroxisome proliferator-activated receptor alpha and carnitine palmitoyl acyl-CoA transferase 1A.
Hepatomegaly
Cholestatic Jaundice Associated with Carnitine Palmitoyltransferase IA Deficiency.
Features of carnitine palmitoyltransferase type I deficiency.
Novel metabolic and molecular findings in hepatic carnitine palmitoyltransferase I deficiency.
The hypolipidemic peroxisome-proliferating drug, bis(carboxymethylthio)-1.10 decane, a dicarboxylic metabolite of tiadenol, is activated to an acylcoenzyme A thioester.
Homocystinuria
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Targeted metabolomic analysis of plasma samples for the diagnosis of inherited metabolic disorders.
hydroxymethylglutaryl-coa lyase deficiency
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Hyperargininemia
Targeted metabolomic analysis of plasma samples for the diagnosis of inherited metabolic disorders.
Hypercholesterolemia
Hypercholesterolemia treatment in a patient with family hypercholesterolemia and myopathy due to carnitine palmitoyltransferase II deficiency with PCSK9 inhibitors.
Hyperglycemia
Malonyl coenzyme A and the regulation of functional carnitine palmitoyltransferase-1 activity and fat oxidation in human skeletal muscle.
Paeoniflorin Protects against Nonalcoholic Fatty Liver Disease Induced by a High-Fat Diet in Mice.
Hyperinsulinism
Hypoglycemia but not hyperglycemia induces rapid changes in pancreatic beta-cell gene transcription.
Malonyl coenzyme A and the regulation of functional carnitine palmitoyltransferase-1 activity and fat oxidation in human skeletal muscle.
Hyperlipidemias
Curcumin attenuates ethanol-induced hepatic steatosis through modulating Nrf2/FXR signaling in hepatocytes.
Paeoniflorin Protects against Nonalcoholic Fatty Liver Disease Induced by a High-Fat Diet in Mice.
[Study on effect of berberine on modulating lipid and CPT I A gene expression].
Hyperparathyroidism
Secondary carnitine palmitoyltransferase deficiency in chronic renal failure and secondary hyperparathyroidism.
Hyperparathyroidism, Secondary
A case of partial carnitine palmitoyltransferase deficiency in a patient undergoing chronic hemodialysis.
Secondary carnitine palmitoyltransferase deficiency in chronic renal failure and secondary hyperparathyroidism.
Hypersensitivity
Mutations of the withered (whd) gene in Drosophila melanogaster confer hypersensitivity to oxidative stress and are lesions of the carnitine palmitoyltransferase I (CPT I) gene.
Hypertension
Niacin improves renal lipid metabolism and slows progression in chronic kidney disease.
Hyperthyroidism
A thyroid hormone response unit formed between the promoter and first intron of the carnitine palmitoyltransferase-Ialpha gene mediates the liver-specific induction by thyroid hormone.
Carnitine palmitoyltransferase: activation and inactivation in liver mitochondria from fed, fasted, hypo- and hyperthyroid rats.
Changes in carnitine palmitoyltransferase-I mRNA abundance produced by hyperthyroidism and hypothyroidism parallel changes in activity.
Differential influences of carnitine palmitoyltransferase-1 inhibition and hyperthyroidism on cardiac growth and sarcoplasmic reticulum phosphorylation.
Expression and activity of lipid and oxidative metabolism enzymes following elevated temperature exposure and thyroid hormone manipulation in juvenile lake whitefish (Coregonus clupeaformis).
Hyperthyroidism facilitates cardiac fatty acid oxidation through altered regulation of cardiac carnitine palmitoyltransferase: studies in vivo and with cardiac myocytes.
The outer carnitine palmitoyltransferase and regulation of fatty acid metabolism in rat liver in different thyroid states.
The relationship between fat synthesis and oxidation in the liver after re-feeding and its regulation by thyroid hormone.
Thyroid hormone regulates carnitine palmitoyltransferase Ialpha gene expression through elements in the promoter and first intron.
Hypertriglyceridemia
Effects of post-suckling n-3 polyunsaturated fatty acids: prevention of dyslipidemia and liver steatosis induced in rats by a sucrose-rich diet during pre- and post-natal life.
Reversal of obesity-induced hypertriglyceridemia by (R)-?-lipoic acid in ZDF (fa/fa) rats.
Hypoglycemia
A Novel Mutation in CPT1A Resulting in Hepatic CPT Deficiency.
A patient with atypical presentation of chronic hepatosteatosis and developmental delay harboring a novel variant in the CPT1A gene.
Abnormal CpG island methylation occurs during in vitro differentiation of human embryonic stem cells.
Carnitine palmitoyltransferase deficiencies.
Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects.
Deficiency of carnitine palmitoyltransferase I.
Functional and structural basis of carnitine palmitoyltransferase 1A deficiency.
Hypoglycemia but not hyperglycemia induces rapid changes in pancreatic beta-cell gene transcription.
Hypothalamic Regulation of Liver and Muscle Nutrient Partitioning by Brain-Specific Carnitine Palmitoyltransferase 1C in Male Mice.
Impaired fasting tolerance among Alaska native children with a common carnitine palmitoyltransferase 1A sequence variant.
Infantile form of carnitine palmitoyltransferase II deficiency with hepatomuscular symptoms and sudden death. Physiopathological approach to carnitine palmitoyltransferase II deficiencies.
Lethal neonatal and severe late infantile forms of carnitine palmitoyltransferase II deficiency associated with compound heterozygosity for different protein truncation mutations.
Naloxone, but not valsartan, preserves responses to hypoglycemia after antecedent hypoglycemia: Role of metabolic reprogramming in counterregulatory failure.
Neonatal hypoglycemia and the CPT1A P479L variant in term newborns: A retrospective cohort study of Inuit newborns from Kivalliq Nunavut.
Normal muscle CPT1 and CPT2 activities in hepatic presentation patients with CPT1 deficiency in fibroblasts. Tissue specific isoforms of CPT1?
Novel metabolic and molecular findings in hepatic carnitine palmitoyltransferase I deficiency.
Novel mutation in carnitine palmitoyltransferase 1A detected through newborn screening for a presymptomatic case in China: a case report.
Novel Mutations in the CPT1A Gene Identified in the Patient Presenting Jaundice as the First Manifestation of Carnitine Palmitoyltransferase 1A Deficiency.
Severe rhabdomyolysis with hypoglycemia in an adult patient with carnitine palmitoyltransferase II deficiency.
The role of chicken ovalbumin upstream promoter transcription factor II in the regulation of hepatic fatty acid oxidation and gluconeogenesis in newborn mice.
[Non-ketotic hypoglycemia caused by carnitine palmitoyl transferase 1 deficiency]
Hypothyroidism
Carnitine palmitoyltransferase in liver and five extrahepatic tissues in the rat. Inhibition by DL-2-bromopalmitoyl-CoA and effect of hypothyroidism.
Changes in carnitine palmitoyltransferase-I mRNA abundance produced by hyperthyroidism and hypothyroidism parallel changes in activity.
Gestational hypothyroidism elicits more pronounced lipid dysregulation in mice than pre-pregnant hypothyroidism.
Triiodothyronine induces lipid oxidation and mitochondrial biogenesis in rat Harderian gland.
Ichthyosis
High frequency of ETFDH c.250G>A mutation in Taiwanese patients with late-onset lipid storage myopathy.
Infections
Acute Respiratory Infection Unveiling CPT II Deficiency.
Acute severe encephalopathy related to human herpesvirus-6 infection in a patient with carnitine palmitoyltransferase 2 deficiency carrying thermolabile variants.
Association of a Genetic Variant of Carnitine Palmitoyltransferase 1A with Infections in Alaska Native Children.
Bilateral supernumerary kidneys: how much is too much?
Carnitine palmitoyl transferase II deficiency in an adolescent presenting with rhabdomyolysis and acute renal failure.
Carnitine palmitoyl transferase II polymorphism is associated with multiple syndromes of acute encephalopathy with various infectious diseases.
Carnitine Palmitoyltransferase 1 Increases Lipolysis, UCP1 Protein Expression and Mitochondrial Activity in Brown Adipocytes.
Enhanced fatty acid oxidation in adipocytes and macrophages reduces lipid-induced triglyceride accumulation and inflammation.
Expression analysis of genes related to lipid metabolism in peripheral blood lymphocytes of chickens challenged with reticuloendotheliosis virus.
Hepatitis C virus infection down-regulates the expression of peroxisome proliferator-activated receptor alpha and carnitine palmitoyl acyl-CoA transferase 1A.
Impaired expression of the peroxisome proliferator-activated receptor alpha during hepatitis C virus infection.
Muscle Carnitine Palmitoyltransferase II Deficiency: A Review of Enzymatic Controversy and Clinical Features.
Pandemic influenza A-associated acute necrotizing encephalopathy without neurologic sequelae.
Rhabdomyolysis and respiratory failure: rare presentation of carnitine palmityl-transferase II deficiency.
Inflammatory Bowel Diseases
Quiescent Endothelial Cells Upregulate Fatty Acid ?-Oxidation for Vasculoprotection via Redox Homeostasis.
Influenza, Human
Pandemic influenza A-associated acute necrotizing encephalopathy without neurologic sequelae.
Insulin Resistance
Age-related susceptibility to insulin resistance arises from a combination of CPT1B decline and lipid overload.
AMPK in the small intestine in normal and pathophysiological conditions.
Brain neuropeptide Y and CCK and peripheral adipokine receptors: temporal response in obesity induced by palatable diet.
Carnitine Palmitoyltransferase 1b Deficiency Protects Mice from Diet-Induced Insulin Resistance.
Carnitine Palmitoyltransferase 1b Deficient Mice Develop Severe Insulin Resistance After Prolonged High Fat Diet Feeding.
Carnitine palmitoyltransferase 2 knockout potentiates palmitate-induced insulin resistance in C2C12 myotubes.
Carnitine palmitoyltransferase-1A prevents free fatty acid induced adipocyte dysfunction through suppression of c-Jun N-terminal kinase.
CPT I overexpression protects L6E9 muscle cells from fatty acid-induced insulin resistance.
Defective hepatic mitochondrial respiratory chain in patients with nonalcoholic steatohepatitis.
Diet Consisting of Balanced Yogurt, Fruit, and Vegetables Modifies the Gut Microbiota and Protects Mice against Nonalcoholic Fatty Liver Disease.
Enhanced susceptibility of Cpt1c knockout mice to glucose intolerance induced by a high-fat diet involves elevated hepatic gluconeogenesis and decreased skeletal muscle glucose uptake.
Exercise Training Improves Whole Body Insulin Resistance via Adiponectin Receptor 1.
Exercise training-induced improvement in skeletal muscle PGC-1?-mediated fat metabolism is independent of dietary glycemic index.
High-intensity exercise to promote accelerated improvements in cardiorespiratory fitness (HI-PACE): study protocol for a randomized controlled trial.
Human skeletal muscle PPARalpha expression correlates with fat metabolism gene expression but not BMI or insulin sensitivity.
Increased reactive oxygen species production and lower abundance of complex I subunits and carnitine palmitoyltransferase 1B protein despite normal mitochondrial respiration in insulin-resistant human skeletal muscle.
Influence of the carnitine palmitoyltransferase inhibitor POCA on myocardial performance and metabolism of insulin resistant rats.
Inhibition of carnitine palmitoyltransferase 1 by phenylalkyloxiranecarboxylic acid and its influence on lipolysis and glucose metabolism in isolated, perfused hearts of streptozotocin-diabetic rats.
Inhibition of carnitine palmitoyltransferase-1 activity alleviates insulin resistance in diet-induced obese mice.
Inhibition of carnitine palymitoyltransferase1b induces cardiac hypertrophy and mortality in mice.
Intramuscular Injection of miR-1 Reduces Insulin Resistance in Obese Mice.
Isolated compounds from Cuscuta pedicellata ameliorate oxidative stress and upregulate expression of some energy regulatory genes in high fat diet induced obesity in rats.
Lack of association of CPT1A polymorphisms or haplotypes on hepatic lipid content or insulin resistance in Japanese individuals with type 2 diabetes mellitus.
Loss of Muscle Carnitine Palmitoyltransferase 2 Prevents Diet-Induced Obesity and Insulin Resistance despite Long-Chain Acylcarnitine Accumulation.
Metabolic characterization of a woman homozygous for the Ser113Leu missense mutation in carnitine palmitoyl transferase II.
Muscle expression of a malonyl-CoA-insensitive carnitine palmitoyltransferase-1 protects mice against high-fat/high-sucrose diet-induced insulin resistance.
Obesity-alleviating potential of asiatic acid and its effects on ACC1, UCP2, and CPT1 mRNA expression in high fat diet-induced obese Sprague-Dawley rats.
Overexpression of carnitine palmitoyltransferase-1 in skeletal muscle is sufficient to enhance fatty acid oxidation and improve high-fat diet-induced insulin resistance.
Palmitate-induced skeletal muscle insulin resistance does not require NF-kappaB activation.
Pathophysiology of type 2 diabetes and modes of action of therapeutic interventions.
Prolonged inhibition of muscle carnitine palmitoyltransferase-1 promotes intramyocellular lipid accumulation and insulin resistance in rats.
Resveratrol improves high fat diet-induced fatty liver and insulin resistance by concomitant inhibiting proteolytic cleavage of SREBPS , FFAs oxidation, and intestinal TGS absorption.
Resveratrol prevents the wasting disorders of mechanical unloading by acting as a physical exercise mimetic in the rat.
Rosiglitazone reduces fatty acid translocase and increases AMPK in skeletal muscle in aged rats: a possible mechanism to prevent high-fat-induced insulin resistance.
Skeletal muscle-specific CPT1 deficiency elevates lipotoxic intermediates but preserves insulin sensitivity.
SUMO-Specific Protease 2 (SENP2) Is an Important Regulator of Fatty Acid Metabolism in Skeletal Muscle.
The hepatokine FGF21 is crucial for peroxisome proliferator-activated receptor-? agonist-induced amelioration of metabolic disorders in obese mice.
The PPARdelta agonist, GW501516, promotes fatty acid oxidation but has no direct effect on glucose utilisation or insulin sensitivity in rat L6 skeletal muscle cells.
Upregulation of peroxisome proliferator-activated receptor gamma coactivator gene (PGC1A) during weight loss is related to insulin sensitivity but not to energy expenditure.
[Effects of fenofibrate on gene expression of carnitine palmitoyltransferase 1 in liver and skeletal muscle and its influence on insulin sensitivity]
Insulinoma
The Interplay of Prolactin and the Glucocorticoids in the Regulation of {beta}-Cell Gene Expression, Fatty Acid Oxidation, and Glucose-Stimulated Insulin Secretion: Implications for Carbohydrate Metabolism in Pregnancy.
Intellectual Disability
Attention remediation following traumatic brain injury in childhood and adolescence.
Ketosis
Carnitine and carnitine palmitoyltransferase in fatty acid oxidation and ketosis.
Carnitine palmitoyltransferase I in liver of periparturient dairy cows: effects of prepartum intake, postpartum induction of ketosis, and periparturient disorders.
Effect of pH on malonyl-CoA inhibition of carnitine palmitoyltransferase I.
Role of carnitine palmitoyltransferase I in the regulation of hepatic ketogenesis during the onset and reversal of chronic diabetes.
The role of changes in the sensitivity of hepatic mitochondrial overt carnitine palmitoyltransferase in determining the onset of the ketosis of starvation in the rat.
Kidney Failure, Chronic
Secondary carnitine palmitoyltransferase deficiency in chronic renal failure and secondary hyperparathyroidism.
Kidney Neoplasms
Identification of CPT1A as a Prognostic Biomarker and Potential Therapeutic Target for Kidney Renal Clear Cell Carcinoma and Establishment of a Risk Signature of CPT1A-Related Genes.
Leukemia
High Expression of CPT1A Predicts Adverse Outcomes: A Potential Therapeutic Target for Acute Myeloid Leukemia.
Targeting the leukemia cell metabolism by the CPT1a inhibition: functional preclinical effects in leukemias.
Therapeutic targeting of HES1 transcriptional programs in T-ALL.
Leukemia, Lymphocytic, Chronic, B-Cell
A reversible carnitine palmitoyltransferase (CPT1) inhibitor offsets the proliferation of chronic lymphocytic leukemia cells.
Leukemia, Myeloid, Acute
High Expression of CPT1A Predicts Adverse Outcomes: A Potential Therapeutic Target for Acute Myeloid Leukemia.
Inhibition of CPT1a as a prognostic marker can synergistically enhance the antileukemic activity of ABT199.
Lipidoses
Hepatic gene expression changes in mouse models with liver-specific deletion or global suppression of the NADPH-cytochrome P450 reductase gene. Mechanistic implications for the regulation of microsomal cytochrome P450 and the fatty liver phenotype.
Liver Cirrhosis
A patient with atypical presentation of chronic hepatosteatosis and developmental delay harboring a novel variant in the CPT1A gene.
Altered serum acylcarnitine profile is associated with the status of nonalcoholic fatty liver disease (NAFLD) and NAFLD-related hepatocellular carcinoma.
The water extract of Sophorae tonkinensis Radix et Rhizoma alleviates non-alcoholic fatty liver disease and its mechanism.
Liver Diseases
?-Tocopherol suppresses hepatic steatosis by increasing CPT-1 expression in a mouse model of diet-induced nonalcoholic fatty liver disease.
Activation of the Peroxisome Proliferator-Activated Receptors (PPAR-?/?) and the Fatty Acid Metabolizing Enzyme Protein CPT1A by Camel Milk Treatment Counteracts the High-Fat Diet-Induced Nonalcoholic Fatty Liver Disease.
Alogliptin alleviates hepatic steatosis in a mouse model of nonalcoholic fatty liver disease by promoting CPT1a expression via Thr172 phosphorylation of AMPK? in the liver.
Ananas comosus L. Leaf Phenols and p-Coumaric Acid Regulate Liver Fat Metabolism by Upregulating CPT-1 Expression.
Association of CYP2C9, CYP2A6, ACSM2A, and CPT1A gene polymorphisms with adverse effects of valproic acid in Chinese patients with epilepsy.
Chinese medicine formula lingguizhugan decoction improves Beta-oxidation and metabolism of Fatty Acid in high-fat-diet-induced rat model of Fatty liver disease.
Cholestatic Jaundice Associated with Carnitine Palmitoyltransferase IA Deficiency.
n-3 Polyunsaturated fatty acids for the management of alcoholic liver disease: A critical review.
TXNIP/VDUP1 attenuates steatohepatitis via autophagy and fatty acid oxidation.
[Effect of chitooligosaccharide on hepatic triglyceride metabolism and related mechanisms].
Liver Diseases, Alcoholic
n-3 Polyunsaturated fatty acids for the management of alcoholic liver disease: A critical review.
Liver Failure
Clinical and genetic characteristics of patients with fatty acid oxidation disorders identified by newborn screening.
long-chain acyl-coa dehydrogenase deficiency
Clinical and genetic characteristics of patients with fatty acid oxidation disorders identified by newborn screening.
Diagnostic assessment and long-term follow-up of 13 patients with Very Long-Chain Acyl-Coenzyme A dehydrogenase (VLCAD) deficiency.
Fatty Acid oxidation disorders in a chinese population in taiwan.
Follow-up of fatty acid ?-oxidation disorders in expanded newborn screening era.
No effect of bezafibrate in patients with CPTII and VLCAD deficiencies.
Rhabdomyolysis with different etiologies in childhood.
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Subclinical effects of long-chain fatty acid ?-oxidation deficiency on the adult heart: A case-control magnetic resonance study.
[Application of tandem mass spectrometry on the diagnosis of fatty acid oxidation disorders]
long-chain-3-hydroxyacyl-coa dehydrogenase deficiency
Differentiation of long-chain fatty acid oxidation disorders using alternative precursors and acylcarnitine profiling in fibroblasts.
Subclinical effects of long-chain fatty acid ?-oxidation deficiency on the adult heart: A case-control magnetic resonance study.
Lymphatic Metastasis
Inhibition of carnitine palmitoyl transferase 1A-induced fatty acid oxidation suppresses cell progression in gastric cancer.
Malnutrition
Impact of protein restriction on the regulation of cardiac carnitine palmitoyltransferase by malonyl-CoA.
Increased L-CPT-1 activity and altered gene expression in pancreatic islets of malnourished adult rats: a possible relationship between elevated free fatty acid levels and impaired insulin secretion.
Pre- and postnatal protein undernutrition increases hepatic carnitine palmitoyltransferase I activity and decreases enzyme sensitivity to inhibitors in the suckling rat.
Undernutrition during suckling in rats elevates plasma adiponectin and its receptor in skeletal muscle regardless of diet composition: a protective effect?
Maple Syrup Urine Disease
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program.
Targeted metabolomic analysis of plasma samples for the diagnosis of inherited metabolic disorders.
medium-chain acyl-coa dehydrogenase deficiency
Fatty Acid oxidation disorders in a chinese population in taiwan.
Follow-up of fatty acid ?-oxidation disorders in expanded newborn screening era.
In vivo stable isotope studies in three patients affected with mitochondrial fatty acid oxidation disorders: limited diagnostic use of 1-13C fatty acid breath test using bolus technique.
Ketogenic diet.
Selective screening for fatty acid oxidation disorders by tandem mass spectrometry: difficulties in practical discrimination.
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program.
[Application of tandem mass spectrometry on the diagnosis of fatty acid oxidation disorders]
[Tandem mass spectrometry analysis and genetic diagnosis of neonates with fatty acid oxidation disorders in central and northern regions of Guangxi].
Melanoma
A fatty acid oxidation-dependent metabolic shift regulates the adaptation of BRAF-mutated melanoma to MAPK inhibitors.
Silencing of Peroxiredoxin 2 and aberrant methylation of 33 CpG islands in putative promoter regions in human malignant melanomas.
Targeting CPT1A enhances metabolic therapy in human melanoma cells with the BRAF V600E mutation.
Memory Disorders
PPAR?/?-agonist GW0742 ameliorates dysfunction in fatty acid oxidation in PSEN1?E9 astrocytes.
Metabolic Diseases
Carbohydrate and fat intake associated with risk of metabolic diseases through epigenetics of CPT1A.
Cystic renal dysplasia as a leading sign of inherited metabolic disease.
Establishing a relationship between prolactin and altered fatty acid ?-oxidation via carnitine palmitoyl transferase 1 in breast cancer cells.
[Alterations of mitochondria in metabolic diseases. Carnitine deficiency, carnitine palmitoyltransferase deficiency and beta oxidation.]
Metabolic Syndrome
A common haplotype of carnitine palmitoyltransferase 1b is associated with the metabolic syndrome.
Association of DNA Methylation at CPT1A Locus with Metabolic Syndrome in the Genetics of Lipid Lowering Drugs and Diet Network (GOLDN) Study.
Genome- and epigenome-wide association study of hypertriglyceridemic waist in Mexican American families.
Metabolic effects of RUBCN/Rubicon deficiency in kidney proximal tubular epithelial cells.
methylcrotonoyl-coa carboxylase deficiency
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program.
Mitochondrial Myopathies
Myopathies due to enzyme deficiencies.
[Exploration of exercise intolerance by 31P NMR spectroscopy of calf muscles coupled with MRI and ergometry]
Multicystic Dysplastic Kidney
SEVERE INFANTILE CARNITINE PALMITOYLTRANSFERASE II (CPT II) DEFICIENCY IN 19-WEEK FETAL SIBS.
Multiple Acyl Coenzyme A Dehydrogenase Deficiency
A survey of Japanese patients with mitochondrial fatty acid beta-oxidation and related disorders as detected from 1985 to 2000.
Cystic renal dysplasia as a leading sign of inherited metabolic disease.
Disorders of fatty acid oxidation and autosomal recessive polycystic kidney disease-different clinical entities and comparable perinatal renal abnormalities.
Fatty Acid oxidation disorders in a chinese population in taiwan.
Follow-up of fatty acid ?-oxidation disorders in expanded newborn screening era.
In vivo stable isotope studies in three patients affected with mitochondrial fatty acid oxidation disorders: limited diagnostic use of 1-13C fatty acid breath test using bolus technique.
Lethal neonatal deficiency of carnitine palmitoyltransferase II associated with dysgenesis of the brain and kidneys.
Therapeutic advances in the management of Pompe disease and other metabolic myopathies.
[Application of tandem mass spectrometry on the diagnosis of fatty acid oxidation disorders]
[Tandem mass spectrometry analysis and genetic diagnosis of neonates with fatty acid oxidation disorders in central and northern regions of Guangxi].
Multiple Carboxylase Deficiency
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Multiple Sclerosis
CPT1A plays a key role in the development and treatment of multiple sclerosis and experimental autoimmune encephalomyelitis.
The distribution of white blood cell fat oxidation in health and disease.
Muscle Cramp
Fuel utilization in subjects with carnitine palmitoyltransferase 2 gene mutations.
[Rhabdomyolysis in carnitine palmitoyltransferase II deficiency: developments in pathophysiology, diagnosis and therapy]
Muscle Hypotonia
Neonatal metabolic myopathies.
Muscle Weakness
Carnitine palmitoyltransferase deficiency in a college athlete: a case report and literature review.
No carnitine palmitoyltransferase deficiency in skeletal muscle in 18 malignant hyperthermia susceptible individuals.
Phenotype of carnitine palmitoyltransferase II (CPT II) deficiency: A questionnaire-based survey.
Stabilization of the thermolabile variant S113L of carnitine palmitoyltransferase II.
Muscular Diseases
A case of partial carnitine palmitoyltransferase deficiency in a patient undergoing chronic hemodialysis.
A variable myopathy associated with heterozygosity for the R503C mutation in the carnitine palmitoyltransferase II gene.
Analysis of lipid profile in lipid storage myopathy.
Biochemical and molecular correlations in carnitine palmitoyltransferase II deficiency.
Carnitine and carnitine palmitoyltransferase in fatty acid oxidation and ketosis.
Carnitine palmitoyltransferase type 2 deficiency: novel mutation in a Native South American family with whole-body muscle magnetic resonance imaging findings: two case reports.
CPT2 gene mutations resulting in lethal neonatal or severe infantile carnitine palmitoyltransferase II deficiency.
Fluxomic assay-assisted diagnosis orientation in a cohort of 11 patients with myopathic form of CPT2 deficiency.
High frequency of ETFDH c.250G>A mutation in Taiwanese patients with late-onset lipid storage myopathy.
Hypercholesterolemia treatment in a patient with family hypercholesterolemia and myopathy due to carnitine palmitoyltransferase II deficiency with PCSK9 inhibitors.
Increased serum lactate dehydrogenase isoenzyme 1 and "flipped" LD-1/LD-2 ratio in myopathy associated with partial carnitine palmitoyltransferase deficiency.
Mutation and biochemical analysis in carnitine palmitoyltransferase type II (CPT II) deficiency.
No carnitine palmitoyltransferase deficiency in skeletal muscle in 18 malignant hyperthermia susceptible individuals.
Partial muscle carnitine palmitoyltransferase-A deficiency. Rhabdomyolysis associated with transiently decreased muscle carnitine content after ibuprofen therapy.
Recurrent Myalgia since Early Infancy-Misleading Clinical Course in a Child with Carnitine Palmitoyltransferase-II Deficiency.
Repeat MRI in acute rhabdomyolysis: correlation with clinicopathological findings.
Rhabdomyolysis and respiratory failure: rare presentation of carnitine palmityl-transferase II deficiency.
Severe rhabdomyolysis in homozygote carnitine palmitoyltransferase II deficiency.
The role of mitochondria in statin-induced myopathy.
Therapeutic advances in the management of Pompe disease and other metabolic myopathies.
[Carnitine palmitoyltransferase deficiency: fatty acid utilization defect causing myopathy]
[Exploration of exercise intolerance by 31P NMR spectroscopy of calf muscles coupled with MRI and ergometry]
[Myopathy due to carnitine palmitoyltransferase deficiency. Report of 2 cases with enzymatic analyses on muscle tissue]
[Myopathy in the course of carnitine palmitoyltransferase II deficiency].
[Rhabdomyolysis due to muscle enzyme deficiencies]
Muscular Dystrophies
Rhabdomyolysis with different etiologies in childhood.
Myalgia
A variable myopathy associated with heterozygosity for the R503C mutation in the carnitine palmitoyltransferase II gene.
Anesthetic management of a parturient with carnitine palmitoyltransferase II deficiency.
Carnitine Palmitoyltransferase II Deficiency (CPT II) Followed By Rhabdomyolysis and Acute Kidney Injury.
Carnitine palmitoyltransferase II deficiency with normal carnitine palmitoyltransferase I in skeletal muscle and leucocytes.
Clinical and biochemical heterogeneity in an Italian family with CPT II deficiency due to Ser 113 Leu mutation.
Clinical features and new molecular findings in Carnitine Palmitoyltransferase II (CPT II) deficiency.
Clinically symptomatic heterozygous carnitine palmitoyltransferase II (CPT II) deficiency.
Disorders of the carnitine cycle and detection by newborn screening.
Exercise tolerance in carnitine palmitoyltransferase II deficiency with IV and oral glucose.
Fuel utilization in subjects with carnitine palmitoyltransferase 2 gene mutations.
Long-term follow-up of bezafibrate treatment in patients with the myopathic form of carnitine palmitoyltransferase 2 deficiency.
Muscle carnitine palmitoyltransferase II deficiency: clinical and molecular genetic features and diagnostic aspects.
Novel mutation in the CPT II gene in a child with periodic febrile myalgia and myoglobinuria.
Novel mutations associated with carnitine palmitoyltransferase II deficiency.
Recurrent myoglobinuria due to carnitine palmitoyltransferase II deficiency: clinical, biochemical, and genetic features of adult-onset cases.
Recurrent rhabdomyolysis caused by carnitine palmitoyltransferase II deficiency, common but under-recognised: Lessons to be learnt.
Severe periodic febrile myalgia in infancy due to carnitine palmitoyltransferase deficiency.
Myocardial Infarction
Single nucleotide polymorphism in CPT1B and CPT2 genes and its association with blood carnitine levels in acute myocardial infarction patients.
Myoglobinuria
A variable myopathy associated with heterozygosity for the R503C mutation in the carnitine palmitoyltransferase II gene.
Anesthetic management of a parturient with carnitine palmitoyltransferase II deficiency.
Biochemical and molecular correlations in carnitine palmitoyltransferase II deficiency.
Carnitine palmitoyltransferase deficiency in a college athlete: a case report and literature review.
Carnitine palmitoyltransferase deficiency: a common cause of recurrent myoglobinuria.
Carnitine Palmitoyltransferase II Deficiency (CPT II) Followed By Rhabdomyolysis and Acute Kidney Injury.
Carnitine palmitoyltransferase II deficiency with normal carnitine palmitoyltransferase I in skeletal muscle and leucocytes.
Carnitine palmitoyltransferase II deficiency: a new cause of recurrent pancreatitis.
Clinical and biochemical heterogeneity in an Italian family with CPT II deficiency due to Ser 113 Leu mutation.
Clinical features and new molecular findings in Carnitine Palmitoyltransferase II (CPT II) deficiency.
Clinical varieties of carnitine and carnitine palmitoyltransferase deficiency.
Coexistence of VHL Disease and CPT2 Deficiency: A Case Report.
Exercise tolerance in carnitine palmitoyltransferase II deficiency with IV and oral glucose.
Exercise-induced recurrent myoglobinuria: defective activity of inner carnitine palmitoyltransferase in muscle mitochondria of two patients.
Fuel utilization in subjects with carnitine palmitoyltransferase 2 gene mutations.
Identification of a common mutation in the carnitine palmitoyltransferase II gene in familial recurrent myoglobinuria patients.
Identification of novel mutations in Spanish patients with muscle carnitine palmitoyltransferase II deficiency.
Malonyl-CoA abnormal inhibition of residual enzyme activity in carnitine palmitoyltransferase deficiency.
Metabolic causes of myoglobinuria.
Metabolic myopathies.
Muscle carnitine palmitoyltransferase II deficiency: clinical and molecular genetic features and diagnostic aspects.
Muscular carnitine palmitoyltransferase II deficiency in infancy.
Neonatal metabolic myopathies.
Normal muscle CPT1 and CPT2 activities in hepatic presentation patients with CPT1 deficiency in fibroblasts. Tissue specific isoforms of CPT1?
Novel mutation in the CPT II gene in a child with periodic febrile myalgia and myoglobinuria.
Novel mutations associated with carnitine palmitoyltransferase II deficiency.
Recurrent myoglobinuria due to carnitine palmitoyltransferase II deficiency: clinical, biochemical, and genetic features of adult-onset cases.
Recurrent myoglobinuria due to carnitine palmitoyltransferase II deficiency: expression of the molecular phenotype in cultured muscle cells.
Recurrent rhabdomyolysis caused by carnitine palmitoyltransferase II deficiency, common but under-recognised: Lessons to be learnt.
Regulatory properties of a mutant carnitine palmitoyltransferase in human skeletal muscle.
Rhabdomyolysis and respiratory failure: rare presentation of carnitine palmityl-transferase II deficiency.
Stabilization of the thermolabile variant S113L of carnitine palmitoyltransferase II.
[Metabolic intolerance to exercise]
[Myoglobinuria and carnitine palmitoyltransferase deficiency. Diagnostic procedure and differential diagnosis]
[Myoglobinuria due to a deficiency of carnitine palmitoyltransferase II. A clinical case report]
[Recurrent myoglobinuria in a girl due to carnitine palmitoyltransferase deficiency]
[Rhabdomyolysis in carnitine palmitoyltransferase II deficiency: developments in pathophysiology, diagnosis and therapy]
[Type II carnitine palmitoyl transferase deficiency complicated by acute respiratory failure]
Myositis
Repeat MRI in acute rhabdomyolysis: correlation with clinicopathological findings.
Narcolepsy
Abnormally low serum acylcarnitine levels in narcolepsy patients.
Evaluation of polygenic risks for narcolepsy and essential hypersomnia.
Genome-wide association studies of sleep disorders.
Identification of the Variations in the CPT1B and CHKB Genes Along with the HLA-DQB1*06:02 Allele in Turkish Narcolepsy Patients and Healthy Persons.
Polymorphism located between CPT1B and CHKB, and HLA-DRB1*1501-DQB1*0602 haplotype confer susceptibility to CNS hypersomnias (essential hypersomnia).
Variant between CPT1B and CHKB associated with susceptibility to narcolepsy.
[Experimental Approach to Analysis of the Relationship between Food Environments and Lifestyle-Related Diseases, Including Cardiac Hypertrophy, Fatty Liver, and Fatigue Symptoms].
Nasopharyngeal Carcinoma
PGC1?/CEBPB/CPT1A axis promotes radiation resistance of nasopharyngeal carcinoma through activating fatty acid oxidation.
Neonatal Sepsis
Differential effects of neonatal endotoxemia on heart and kidney carnitine palmitoyl transferase I.
Myocardial carnitine palmitoyltransferase I as a target for oxidative modification in inflammation and sepsis.
Neoplasm Metastasis
Carnitine palmitoyl transferase 1A is a novel diagnostic and predictive biomarker for breast cancer.
Carnitine palmitoyl transferase-1A (CPT1A): a new tumor specific target in human breast cancer.
Carnitine palmitoyltransferase 1A (CPT1A): a transcriptional target of PAX3-FKHR and mediates PAX3-FKHR-dependent motility in alveolar rhabdomyosarcoma cells.
CPT1A regulates breast cancer-associated lymphangiogenesis via VEGF signaling.
Development of a Genetic and Clinical Data-Based (GC) Risk Score for Predicting Survival of Hepatocellular Carcinoma Patients After Tumor Resection.
Inhibition of carnitine palmitoyl transferase 1A-induced fatty acid oxidation suppresses cell progression in gastric cancer.
Multi-omics Integration Analysis Robustly Predicts High-Grade Patient Survival and Identifies CPT1B Effect on Fatty Acid Metabolism in Bladder Cancer.
Neoplasms
A novel miR-1291-ERR?-CPT1C axis modulates tumor cell proliferation, metabolism and tumorigenesis.
A sib-pair analysis study of 15 candidate genes in French families with morbid obesity: indication for linkage with islet 1 locus on chromosome 5q.
Adzuki bean ameliorates hepatic lipogenesis and proinflammatory mediator expression in mice fed a high-cholesterol and high-fat diet to induce nonalcoholic fatty liver disease.
Alisol A 24-Acetate Prevents Hepatic Steatosis and Metabolic Disorders in HepG2 Cells.
Anti-Obesity Effect of T. Chebula Fruit Extract on High Fat Diet Induced Obese Mice: A Possible Alternative Therapy.
Biolabel-led research pattern positions the effects and mechanisms of Sophorae Tonkinensis radix et rhizome on lung diseases: A novel strategy for computer-aided herbal medicine research based on omics and bioinformatics.
C75 increases peripheral energy utilization and fatty acid oxidation in diet-induced obesity.
Cardiac metabolism, inflammation, and peroxisome proliferator-activated receptors modulated by 1,25-dihydroxyvitamin D3 in diabetic rats.
Carnitine palmitoyl transferase 1A is a novel diagnostic and predictive biomarker for breast cancer.
Carnitine palmitoyl transferase-1A (CPT1A): a new tumor specific target in human breast cancer.
Carnitine palmitoyltransferase 1 A expression profile in canine mammary tumors.
Carnitine palmitoyltransferase 1A functions to repress FoxO transcription factors to allow cell cycle progression in ovarian cancer.
Carnitine palmitoyltransferase 1C promotes cell survival and tumor growth under conditions of metabolic stress.
Carnitine palmitoyltransferase 1C regulates cancer cell senescence through mitochondria-associated metabolic reprograming.
Carnitine palmitoyltransferase 1C reverses cellular senescence of MRC-5 fibroblasts via regulating lipid accumulation and mitochondrial function.
Carnitine palmitoyltransferase 1C: From cognition to cancer.
Carnitine palmitoyltransferase II activity is decreased in liver mitochondria of cachectic rats bearing the Walker 256 carcinosarcoma: effect of indomethacin treatment.
CPT1A and fatty acid ?-oxidation are essential for tumor cell growth and survival in hormone receptor-positive breast cancer.
CPT1A Supports Castration-Resistant Prostate Cancer in Androgen-Deprived Conditions.
CPT1A-mediated Fat Oxidation, Mechanisms, and Therapeutic Potential.
CPT1C promotes human mesenchymal stem cells survival under glucose deprivation through the modulation of autophagy.
Defective hepatic mitochondrial respiratory chain in patients with nonalcoholic steatohepatitis.
Depletion of the novel p53-target gene carnitine palmitoyltransferase 1C delays tumor growth in the neurofibromatosis type I tumor model.
Development of a Genetic and Clinical Data-Based (GC) Risk Score for Predicting Survival of Hepatocellular Carcinoma Patients After Tumor Resection.
Down-regulation of the phosphoenolpyruvate carboxykinase gene in human colon tumors and induction by omega-3 fatty acids.
Effects of bezafibrate, PPAR pan-agonist, and GW501516, PPARdelta agonist, on development of steatohepatitis in mice fed a methionine- and choline-deficient diet.
Effects of carnitine palmitoyltransferases on cancer cellular senescence.
Elevated expression of fatty acid synthase and nuclear localization of carnitine palmitoyltransferase 1C are common among human gliomas.
Enhancing cancer-associated fibroblast fatty acid catabolism within a metabolically challenging tumor microenvironment drives colon cancer peritoneal metastasis.
Expression of Lipid Metabolism-Related Proteins Differs between Invasive Lobular Carcinoma and Invasive Ductal Carcinoma.
Expression of lipid metabolism-related proteins in breast phyllodes tumors.
Fatty acid oxidation and carnitine palmitoyltransferase I: emerging therapeutic targets in cancer.
Fatty acid oxidation is associated with proliferation and prognosis in breast and other cancers.
Fatty acid synthase inhibition in human breast cancer cells leads to malonyl-CoA-induced inhibition of fatty acid oxidation and cytotoxicity.
Fatty acid synthase inhibition triggers apoptosis during S phase in human cancer cells.
Functional genomic characterization of delipidation elicited by trans-10, cis-12-conjugated linoleic acid (t10c12-CLA) in a polygenic obese line of mice.
Gamma-linolenic acid alters the composition of mitochondrial membrane subfractions, decreases outer mitochondrial membrane binding of hexokinase and alters carnitine palmitoyltransferase I properties in the Walker 256 rat tumour.
Genetic alterations in fatty acid transport and metabolism genes are associated with metastatic progression and poor prognosis of human cancers.
Genetics and molecular biology: fatty acid metabolism in cancer cell survival; carnitine palmitoyltransferase-1 as a critical anticancer target.
Glioblastoma Utilizes Fatty Acids and Ketone Bodies for Growth Allowing Progression during Ketogenic Diet Therapy.
HIF drives lipid deposition and cancer in ccRCC via repression of fatty acid metabolism.
High Expression of CPT1A Predicts Adverse Outcomes: A Potential Therapeutic Target for Acute Myeloid Leukemia.
High-fat diet-activated fatty acid oxidation mediates intestinal stemness and tumorigenicity.
Human and rat tumour cells possess mitochondrial carnitine palmitoyltransferase I and II: effects of insulin.
Identification of CPT1A as a Prognostic Biomarker and Potential Therapeutic Target for Kidney Renal Clear Cell Carcinoma and Establishment of a Risk Signature of CPT1A-Related Genes.
Identifying off-target effects of etomoxir reveals that carnitine palmitoyltransferase I is essential for cancer cell proliferation independent of ?-oxidation.
Immunodetection of rat Walker 256 tumour mitochondrial carnitine palmitoyltransferase I and II: evidence for the control of CPT II expression by insulin.
In vivo inhibition of Walker 256 tumour carnitine palmitoyltransferase I by soya oil dietary supplementation.
Induction of apoptosis by polyunsaturated fatty acids and its relationship to fatty acid inhibition of carnitine palmitoyltransferase I activity in Hep2 cells.
Influence of DMBA-induced mammary cancer on the liver CPT I, mit HMG-CoA synthase and PPARalpha mRNA expression in rats fed a low or high corn oil diet.
Inhibition of carnitine palmitoyl transferase 1A-induced fatty acid oxidation suppresses cell progression in gastric cancer.
Inhibition of CPT1a as a prognostic marker can synergistically enhance the antileukemic activity of ABT199.
Intracellular mechanisms underlying lipid accumulation (white opaque substance) in gastric epithelial neoplasms: A pilot study of expression profiles of lipid-metabolism-associated genes.
iRGD-modified exosomes effectively deliver CPT1A siRNA to colon cancer cells, reversing oxaliplatin resistance by regulating fatty acid oxidation.
L-carnitine ameliorates cancer cachexia in mice by regulating the expression and activity of carnitine palmityl transferase.
Lipid catabolism inhibition sensitizes prostate cancer cells to antiandrogen blockade.
Lipidomics reveals carnitine palmitoyltransferase 1C protects cancer cells from lipotoxicity and senescence.
Loss of tumor suppressive properties of lipid metabolism enzyme CPT2 in ovarian carcinoma: Comment on "CPT2 down-regulation promotes tumor growth and metastasis through inducing ROS/NF?B pathway in ovarian cancer" by Zhang et al.
Mechanism of apoptosis induced by the inhibition of fatty acid synthase in breast cancer cells.
Metabolic fate and effects of saturated and unsaturated fatty acids in Hep2 human larynx tumor cells.
Metabolic Pathway Signatures Associated with Urinary Metabolite Biomarkers Differentiate Bladder Cancer Patients from Healthy Controls.
Metabolic rewiring of macrophages by CpG potentiates clearance of cancer cells and overcomes tumor-expressed CD47-mediated 'don't-eat-me' signal.
Molecular Pathways: Tumors cells co-opt the brain-specific metabolism gene CPT1C to promote survival.
Multi-omics Integration Analysis Robustly Predicts High-Grade Patient Survival and Identifies CPT1B Effect on Fatty Acid Metabolism in Bladder Cancer.
Myocardial expression profiles of candidate molecules in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia compared to those with dilated cardiomyopathy and healthy controls.
n-3 Polyunsaturated fatty acids for the management of alcoholic liver disease: A critical review.
N6-methyladenosine-induced ERR? triggers chemoresistance of cancer cells through upregulation of ABCB1 and metabolic reprogramming.
Nobiletin improves obesity and insulin resistance in high-fat diet-induced obese mice.
PGC-1? activator-induced fatty acid oxidation in tumor-infiltrating CTLs enhances effects of PD-1 blockade therapy in lung cancer.
Polymorphisms in CPT1B and CPT2 have no significant effect on plasma carnitine levels in Japanese cancer patients.
PPAR? regulates tumor cell proliferation and senescence via a novel target gene carnitine palmitoyltransferase 1C.
Prognostic Value of the Overexpression of Fatty Acid Metabolism-Related Enzymes in Squamous Cell Carcinoma of the Head and Neck.
Regulation of tumour cell fatty acid oxidation by n-6 polyunsaturated fatty acids.
Significance of thymidine phosphorylase in metronomic chemotherapy using CPT-11 and doxifluridine for advanced colorectal carcinoma.
Targeting CPT1A-mediated fatty acid oxidation sensitizes nasopharyngeal carcinoma to radiation therapy.
Targeting CPT1B as a potential therapeutic strategy in castration-resistant and enzalutamide-resistant prostate cancer.
Targeting Fat Oxidation in Mouse Prostate Cancer Decreases Tumor Growth and Stimulates Anti-Cancer Immunity.
The Proteome of Primary Prostate Cancer.
Tumor-induced alterations in hepatic malic enzyme and carnitine palmitoyltransferase activity.
TXNIP/VDUP1 attenuates steatohepatitis via autophagy and fatty acid oxidation.
Upregulation of CPT1A is essential for the tumor-promoting effect of adipocytes in colon cancer.
Nervous System Malformations
Lethal neonatal deficiency of carnitine palmitoyltransferase II associated with dysgenesis of the brain and kidneys.
Neurofibromatoses
Depletion of the novel p53-target gene carnitine palmitoyltransferase 1C delays tumor growth in the neurofibromatosis type I tumor model.
Neurofibromatosis 1
Depletion of the novel p53-target gene carnitine palmitoyltransferase 1C delays tumor growth in the neurofibromatosis type I tumor model.
Neuroleptic Malignant Syndrome
Genetic analysis of ryanodine receptor 1 gene and carnitine palmitoyltransferase II gene: An autopsy case of neuroleptic malignant syndrome related to vegetamin.
Non-alcoholic Fatty Liver Disease
?-Tocopherol suppresses hepatic steatosis by increasing CPT-1 expression in a mouse model of diet-induced nonalcoholic fatty liver disease.
Activation of the Peroxisome Proliferator-Activated Receptors (PPAR-?/?) and the Fatty Acid Metabolizing Enzyme Protein CPT1A by Camel Milk Treatment Counteracts the High-Fat Diet-Induced Nonalcoholic Fatty Liver Disease.
Alogliptin alleviates hepatic steatosis in a mouse model of nonalcoholic fatty liver disease by promoting CPT1a expression via Thr172 phosphorylation of AMPK? in the liver.
Beneficial effects of neomangiferin on high fat diet-induced nonalcoholic fatty liver disease in rats.
Gan-Jiang-Ling-Zhu decoction alleviates hepatic steatosis in rats by the miR-138-5p/CPT1B axis.
Genome-wide analysis of DNA methylation in human peripheral leukocytes identifies potential biomarkers of nonalcoholic fatty liver disease.
Gynostemma pentaphyllum Attenuates the Progression of Nonalcoholic Fatty Liver Disease in Mice: A Biomedical Investigation Integrated with In Silico Assay.
Liver CPT1A gene therapy reduces diet-induced hepatic steatosis in mice and highlights potential lipid biomarkers for human NAFLD.
n-3 Polyunsaturated fatty acids for the management of alcoholic liver disease: A critical review.
Oxymatrine attenuates hepatic steatosis in non-alcoholic fatty liver disease rats fed with high fructose diet through inhibition of sterol regulatory element binding transcription factor 1 (Srebf1) and activation of peroxisome proliferator activated receptor alpha (Ppar?).
Resveratrol improves high fat diet-induced fatty liver and insulin resistance by concomitant inhibiting proteolytic cleavage of SREBPS , FFAs oxidation, and intestinal TGS absorption.
Study on the protection of water extracts of Polygoni Multiflori Radix and Polygoni Multiflori Radix Praeparata against NAFLD and its mechanism.
The water extract of Sophorae tonkinensis Radix et Rhizoma alleviates non-alcoholic fatty liver disease and its mechanism.
TXNIP/VDUP1 attenuates steatohepatitis via autophagy and fatty acid oxidation.
[Effect of chitooligosaccharide on hepatic triglyceride metabolism and related mechanisms].
Obesity
A sib-pair analysis study of 15 candidate genes in French families with morbid obesity: indication for linkage with islet 1 locus on chromosome 5q.
Anti-Obesity Effects of Poly-?-glutamic Acid with or without Isoflavones on High-Fat Diet Induced Obese Mice.
Blood Cells as a Source of Transcriptional Biomarkers of Childhood Obesity and Its Related Metabolic Alterations: Results of the IDEFICS Study.
C75 increases peripheral energy utilization and fatty acid oxidation in diet-induced obesity.
C75 [4-methylene-2-octyl-5-oxo-tetrahydro-furan-3-carboxylic acid] activates carnitine palmitoyltransferase-1 in isolated mitochondria and intact cells without displacement of bound malonyl CoA.
Canagliflozin ameliorates obesity by improving mitochondrial function and fatty acid oxidation via PPAR? in vivo and in vitro.
Carnitine palmitoyltransferase 1B 531K allele carriers sustain a higher respiratory quotient after aerobic exercise, but ?3-adrenoceptor 64R allele does not affect lipolysis: a human model.
Chronic administration of recombinant IL-6 upregulates lipogenic enzyme expression and aggravates high-fat-diet-induced steatosis in IL-6-deficient mice.
CPT1C in the ventromedial nucleus of the hypothalamus is necessary for brown fat thermogenesis activation in obesity.
Differential epigenetic and transcriptional response of the skeletal muscle carnitine palmitoyltransferase 1B (CPT1B) gene to lipid exposure with obesity.
Effect of Chronic CPT-1 Inhibition on Myocardial Ischemia-Reperfusion Injury (I/R) in a Model of Diet-Induced Obesity.
Effects of hypoxic exercise training on microRNA expression and lipid metabolism in obese rat livers.
Enhanced susceptibility of Cpt1c knockout mice to glucose intolerance induced by a high-fat diet involves elevated hepatic gluconeogenesis and decreased skeletal muscle glucose uptake.
Examination of carnitine palmitoyltransferase 1 abundance in white adipose tissue: implications in obesity research.
Genes involved in fatty acid partitioning and binding, lipolysis, monocyte/macrophage recruitment, and inflammation are overexpressed in the human fatty liver of insulin-resistant subjects.
Genetic polymorphisms in carnitine palmitoyltransferase 1A gene are associated with variation in body composition and fasting lipid traits in Yup'ik Eskimos.
Glial ?-oxidation regulates Drosophila energy metabolism.
Hypothalamic ceramide levels regulated by CPT1C mediate the orexigenic effect of ghrelin.
Hypothalamic malonyl-CoA and CPT1c in the treatment of obesity.
Hypothalamic Regulation of Liver and Muscle Nutrient Partitioning by Brain-Specific Carnitine Palmitoyltransferase 1C in Male Mice.
Impaired oxidative capacity due to decreased CPT1b levels as a contributing factor to fat accumulation in obesity.
Influence of breastfeeding on blood-cell transcript-based biomarkers of health in children.
Lipid oxidation is reduced in obese human skeletal muscle.
Liver CPT1A gene therapy reduces diet-induced hepatic steatosis in mice and highlights potential lipid biomarkers for human NAFLD.
Loss of Muscle Carnitine Palmitoyltransferase 2 Prevents Diet-Induced Obesity and Insulin Resistance despite Long-Chain Acylcarnitine Accumulation.
Metabolism as a tool for understanding human brain evolution: lipid energy metabolism as an example.
Methylation loci associated with body mass index, waist circumference, and waist-to-hip ratio in Chinese adults: an epigenome-wide analysis.
Modulation of carnitine palmitoyltransferase-1 for the treatment of obesity.
Molecular therapy for obesity and diabetes based on a long-term increase in hepatic fatty-acid oxidation.
Ontogeny of hepatic energy metabolism genes in mice as revealed by RNA-sequencing.
Poly-ion complex micelles effectively deliver CoA-conjugated CPT1A inhibitors to modulate lipid metabolism in brain cells.
Positive regulation of hepatic carnitine palmitoyl transferase 1A (CPT1A) activities by soy isoflavones and L-carnitine.
SUMO-Specific Protease 2 (SENP2) Is an Important Regulator of Fatty Acid Metabolism in Skeletal Muscle.
The brain-specific carnitine palmitoyltransferase-1c regulates energy homeostasis.
The distribution of white blood cell fat oxidation in health and disease.
The hepatokine FGF21 is crucial for peroxisome proliferator-activated receptor-? agonist-induced amelioration of metabolic disorders in obese mice.
The impact of CPT1B rs470117, LEPR rs1137101 and BDNF rs6265 polymorphisms on the risk of developing obesity in an Italian population.
The Loss Of Macrophage Fatty Acid Oxidation Does Not Potentiate Systemic Metabolic Dysfunction.
Theobromine ameliorates nonalcoholic fatty liver disease by regulating hepatic lipid metabolism via mTOR signaling pathway in vivo and in vitro.
Therapeutic promises of Chlorogenic acid with special emphasis on its anti-obesity property.
Ultra-fast simultaneous detection of obesity-related coenzymes in mice using microchip electrophoresis with a LIF detector.
Use of human PBMC to analyse the impact of obesity on lipid metabolism and metabolic status: a proof-of-concept pilot study.
Validation of BMI genetic risk score and DNA methylation in a Korean population.
Variants within the muscle and liver isoforms of the carnitine palmitoyltransferase I (CPT1) gene interact with fat intake to modulate indices of obesity in French-Canadians.
Obesity, Morbid
Differential epigenetic and transcriptional response of the skeletal muscle carnitine palmitoyltransferase 1B (CPT1B) gene to lipid exposure with obesity.
ornithine carbamoyltransferase deficiency
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Targeted metabolomic analysis of plasma samples for the diagnosis of inherited metabolic disorders.
Ornithine Carbamoyltransferase Deficiency Disease
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Targeted metabolomic analysis of plasma samples for the diagnosis of inherited metabolic disorders.
Osteoporosis
Functional relevance for associations between osteoporosis and genetic variants.
Otitis Media
Association of the CPT1A p.P479L Metabolic Gene Variant With Childhood Respiratory and Other Infectious Illness in Nunavut.
Ovarian Neoplasms
Carnitine palmitoyltransferase 1A functions to repress FoxO transcription factors to allow cell cycle progression in ovarian cancer.
Overweight
Blood Cells as a Source of Transcriptional Biomarkers of Childhood Obesity and Its Related Metabolic Alterations: Results of the IDEFICS Study.
Pancreatic Neoplasms
A novel miR-1291-ERR?-CPT1C axis modulates tumor cell proliferation, metabolism and tumorigenesis.
Pancreatitis
Carnitine palmitoyltransferase II deficiency: a new cause of recurrent pancreatitis.
pantoate-beta-alanine ligase (amp-forming) deficiency
Targeted metabolomic analysis of plasma samples for the diagnosis of inherited metabolic disorders.
Paraplegia
Mutation in CPT1C Associated With Pure Autosomal Dominant Spastic Paraplegia.
Paresis
Clinically symptomatic heterozygous carnitine palmitoyltransferase II (CPT II) deficiency.
peptidyl-glutamate 4-carboxylase deficiency
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Peripheral Nervous System Diseases
Mechanisms of peripheral neuropathy associated with bortezomib and vincristine in patients with newly diagnosed multiple myeloma: a prospective analysis of data from the HOVON-65/GMMG-HD4 trial.
Trifunctional enzyme deficiency: adult presentation of a usually fatal beta-oxidation defect.
Phenylketonurias
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program.
Placenta Accreta
An unusual case of placenta increta in a Carnitine palmitoyltransferase deficiency carrier.
Polycystic Kidney Diseases
Carnitine palmitoyltransferase II deficiency in a prenatal case with polycystic kidney disease-like phenotype.
Polymyositis
Repeat MRI in acute rhabdomyolysis: correlation with clinicopathological findings.
Porphyrias
Ketogenic diet.
Pregnancy Complications
High glucose levels reduce fatty acid oxidation and increase triglyceride accumulation in human placenta.
Serious pregnancy complications in a patient with previously undiagnosed carnitine palmitoyltransferase 1 deficiency.
Propionic Acidemia
Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program.
Targeted metabolomic analysis of plasma samples for the diagnosis of inherited metabolic disorders.
Prostatic Neoplasms
Cationic Polymer Nanoparticles-Mediated Delivery of miR-124 Impairs Tumorigenicity of Prostate Cancer Cells.
CPT1A Over-Expression Increases Reactive Oxygen Species in the Mitochondria and Promotes Antioxidant Defenses in Prostate Cancer.
CPT1A Supports Castration-Resistant Prostate Cancer in Androgen-Deprived Conditions.
Deregulation of MicroRNAs mediated control of carnitine cycle in prostate cancer: molecular basis and pathophysiological consequences.
Targeting CPT1B as a potential therapeutic strategy in castration-resistant and enzalutamide-resistant prostate cancer.
Targeting prostate cancer cell metabolism: impact of hexokinase and CPT-1 enzymes.
[A multi-omics study on NKX3.1-related node genes in prostate cancer].
Protein Deficiency
Differentiation of long-chain fatty acid oxidation disorders using alternative precursors and acylcarnitine profiling in fibroblasts.
Neonatal metabolic myopathies.
Proteinuria
High-calorie diet partially ameliorates dysregulation of intrarenal lipid metabolism in remnant kidney.
Niacin improves renal lipid metabolism and slows progression in chronic kidney disease.
Psoriasis
A new therapeutic approach to treat psoriasis by inhibition of fatty acid oxidation by Etomoxir.
pyruvate carboxylase deficiency
Ketogenic diet.
Pyruvate Carboxylase Deficiency Disease
Ketogenic diet.
pyruvate decarboxylase deficiency
[Lipid storage myopathies--carnitine deficiency, carnitine palmitoyltransferase deficiency, pyruvate decarboxylase deficiency]
Pyruvate Dehydrogenase Complex Deficiency Disease
[Lipid storage myopathies--carnitine deficiency, carnitine palmitoyltransferase deficiency, pyruvate decarboxylase deficiency]
Renal Insufficiency
[Rhabdomyolysis caused by carnitine palmitoyltransferase deficiency. Prevention of acute kidney failure]
Reperfusion Injury
Effect of Chronic CPT-1 Inhibition on Myocardial Ischemia-Reperfusion Injury (I/R) in a Model of Diet-Induced Obesity.
Reducing the Burden of Ischemia Reperfusion : Editorial to: "Effect of Chronic CPT-1 Inhibition on Myocardial Ischemia-Reperfusion Injury (I/R) in a Model of Diet-Induced Obesity" by G. Maarman et al.
Respiratory Insufficiency
Partial muscle carnitine palmitoyltransferase-A deficiency. Rhabdomyolysis associated with transiently decreased muscle carnitine content after ibuprofen therapy.
Rhabdomyolysis and respiratory failure: rare presentation of carnitine palmityl-transferase II deficiency.
[Acute respiratory insufficiency and carnitine palmitoyltransferase deficiency]
[Type II carnitine palmitoyl transferase deficiency complicated by acute respiratory failure]
Restless Legs Syndrome
Genome-wide association studies of sleep disorders.
Retinoblastoma
Microarray analysis of perichondral and reserve growth plate zones identifies differential gene expressions and signal pathways.
Reye Syndrome
Carnitine palmitoyltransferase 1A deficiency: abnormal muscle biopsy findings in a child presenting with Reye's syndrome.
Defects in activation and transport of fatty acids.
Expression analysis of two mutations in carnitine palmitoyltransferase IA deficiency.
Rhabdomyolysis
A variable myopathy associated with heterozygosity for the R503C mutation in the carnitine palmitoyltransferase II gene.
Acute renal failure due to carnitine palmitoyltransferase deficiency.
Acute renal failure due to carnitine palmitoyltransferase II deficiency.
Acute Respiratory Infection Unveiling CPT II Deficiency.
An ignored cause of red urine in children: rhabdomyolysis due to carnitine palmitoyltransferase II (CPT-II) deficiency.
Anesthetic management of a parturient with carnitine palmitoyltransferase II deficiency.
Bilateral supernumerary kidneys: how much is too much?
Biochemical evidence for heterozygosity in muscular carnitine palmitoyltransferase deficiency.
Carnitine palmitoyl transferase II deficiency in an adolescent presenting with rhabdomyolysis and acute renal failure.
Carnitine palmitoyltransferase deficiency in a college athlete: a case report and literature review.
Carnitine palmitoyltransferase deficiency in pregnancy.
Carnitine palmitoyltransferase deficiency: an underdiagnosed condition?
Carnitine Palmitoyltransferase II Deficiency (CPT II) Followed By Rhabdomyolysis and Acute Kidney Injury.
Carnitine palmitoyltransferase II deficiency and post-COVID vaccination rhabdomyolysis.
Carnitine palmitoyltransferase II deficiency due to a novel gene variant in a patient with rhabdomyolysis and ARF.
Carnitine palmitoyltransferase II deficiency: successful anaplerotic diet therapy.
Cause of recurrent rhabdomyolysis, carnitine palmitoyltransferase II deficiency and novel pathogenic mutation.
Clinical and genetic characteristics of patients with fatty acid oxidation disorders identified by newborn screening.
Clinically symptomatic heterozygous carnitine palmitoyltransferase II (CPT II) deficiency.
Defects in activation and transport of fatty acids.
Diagnostic assessment and long-term follow-up of 13 patients with Very Long-Chain Acyl-Coenzyme A dehydrogenase (VLCAD) deficiency.
Disorders of the carnitine cycle and detection by newborn screening.
Exome-Based Rare-Variant Analyses in CKD.
Fatty acid oxidation disorders: outcome and long-term prognosis.
Fenofibrate therapy in carnitine palmitoyl transferase type 2 deficiency.
First Japanese Case of Carnitine Palmitoyltransferase II Deficiency with the Homozygous Point Mutation S113L.
Functional analysis of iPSC-derived myocytes from a patient with carnitine palmitoyltransferase II deficiency.
Genetic analysis of ryanodine receptor 1 gene and carnitine palmitoyltransferase II gene: An autopsy case of neuroleptic malignant syndrome related to vegetamin.
Heterogeneity of carnitine-palmitoyltransferase deficiency.
Long-term follow-up of bezafibrate treatment in patients with the myopathic form of carnitine palmitoyltransferase 2 deficiency.
Macro creatine kinase in a case of carnitine palmitoyltransferase deficiency.
No carnitine palmitoyltransferase deficiency in skeletal muscle in 18 malignant hyperthermia susceptible individuals.
Partial muscle carnitine palmitoyltransferase-A deficiency. Rhabdomyolysis associated with transiently decreased muscle carnitine content after ibuprofen therapy.
Postinfectious Rhabdomyolysis in a 5-Year-Old Boy: When to Look a Little Deeper.
Recurrent rhabdomyolysis associated with influenza-like illness in a weight-lifter.
Recurrent rhabdomyolysis caused by carnitine palmitoyltransferase II deficiency, common but under-recognised: Lessons to be learnt.
Repeated and progressive rhabdomyolysis due to a novel carnitine palmitoyltransferase II gene variant in an adult male: A case report.
Rhabdomyolysis and Cardiomyopathy in a 20-Year-Old Patient with CPT II Deficiency.
Rhabdomyolysis and respiratory failure: rare presentation of carnitine palmityl-transferase II deficiency.
Rhabdomyolysis caused by carnitine palmitoyltransferase 2 deficiency: A case report and systematic review of the literature.
Rhabdomyolysis with different etiologies in childhood.
Severe rhabdomyolysis in homozygote carnitine palmitoyltransferase II deficiency.
Severe rhabdomyolysis with hypoglycemia in an adult patient with carnitine palmitoyltransferase II deficiency.
Unmasking of carnitine palmitoyltransferase deficiency during an acute exacerbation of asthma complicated by rhabdomyolysis in a soldier.
Valproic acid triggers acute rhabdomyolysis in a patient with carnitine palmitoyltransferase type II deficiency.
[Abnormal regulation of carnitine palmitoyltransferase in monozygotic twins as the cause of rhabdomyolysis]
[Acute renal insufficiency caused by rhabdomyolysis due to deficiency of carnitine palmitoyltransferase]
[Carnitine palmitoyltransferase deficiency as the cause of effort rhabdomyolysis]
[Rhabdomyolysis caused by carnitine palmitoyltransferase deficiency. Prevention of acute kidney failure]
[Rhabdomyolysis caused by carnitine palmitoyltransferase deficiency]
[Rhabdomyolysis due to muscle enzyme deficiencies]
[Rhabdomyolysis in carnitine palmitoyltransferase deficiency]
[Rhabdomyolysis in carnitine palmitoyltransferase II deficiency: developments in pathophysiology, diagnosis and therapy]
Rhabdomyosarcoma, Alveolar
Carnitine palmitoyltransferase 1A (CPT1A): a transcriptional target of PAX3-FKHR and mediates PAX3-FKHR-dependent motility in alveolar rhabdomyosarcoma cells.
Riboflavin Deficiency
Hepatic mitochondrial and peroxisomal oxidative capacity in riboflavin deficiency: effect of age, dietary fat and starvation in rats.
Transcriptional regulation of carnitine palmitoyltransferase synthesis in riboflavin deficiency in rats.
Sarcoma
Tumor-induced alterations in hepatic malic enzyme and carnitine palmitoyltransferase activity.
Seizures
Carnitine palmitoyltransferase 1A (CPT1A) P479L prevalence in live newborns in Yukon, Northwest Territories, and Nunavut.
Features of carnitine palmitoyltransferase type I deficiency.
Normal muscle CPT1 and CPT2 activities in hepatic presentation patients with CPT1 deficiency in fibroblasts. Tissue specific isoforms of CPT1?
Sepsis
Accelerated hepatic lipid synthesis in fasted septic rats.
Differential effects of neonatal endotoxemia on heart and kidney carnitine palmitoyl transferase I.
Hepatic gene expression and cytokine responses to sterile inflammation: comparison with cecal ligation and puncture sepsis in the rat.
Myocardial carnitine palmitoyltransferase I as a target for oxidative modification in inflammation and sepsis.
Rhabdomyolysis with different etiologies in childhood.
The Ca2+ second messenger system and interleukin-1-alpha modulation of hepatic gene transcription and mitochondrial fat oxidation.
The effect of surgical treatment following peritoneal sepsis on hepatic gene expression.
The possible inhibitory role of the leucine-zipper DNA binding protein c-fos in the regulation of hepatic gene expression after sepsis.
short-chain acyl-coa dehydrogenase deficiency
Selective screening for inborn errors of metabolism on clinical patients using tandem mass spectrometry in China: a four-year report.
Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program.
[Application of tandem mass spectrometry on the diagnosis of fatty acid oxidation disorders]
[Tandem mass spectrometry analysis and genetic diagnosis of neonates with fatty acid oxidation disorders in central and northern regions of Guangxi].
Sickle Cell Trait
Postoperative coma in a child with carnitine palmitoyltransferase I deficiency.
Sleep Deprivation
Blood-gene expression reveals reduced circadian rhythmicity in individuals resistant to sleep deprivation.
Central AMPK contributes to sleep homeostasis in mice.
Spastic Paraplegia, Hereditary
A novel CPT1C variant causes pure hereditary spastic paraplegia with benign clinical course.
Carnitine palmitoyltransferase 1C: From cognition to cancer.
Starvation
CPT1C promotes human mesenchymal stem cells survival under glucose deprivation through the modulation of autophagy.
Cycloheximide blocks changes in rat liver carnitine palmitoyltransferase 1 activity in starvation.
Dietary fatty acids influence the activity and metabolic control of mitochondrial carnitine palmitoyltransferase I in rat heart and skeletal muscle.
Differential regulation of hepatic gene expression by starvation versus refeeding following a high-sucrose or high-fat diet.
Effect of diet and starvation on hepatic mitochondrial function in the rat.
Effect of dietary ascorbate on lipogenesis and lipolysis activities in black sea bream, Acanthopagrus schlegelii.
Effect of insulin on the properties of liver carnitine palmitoyltransferase in the starved rat: assessment by the euglycemic hyperinsulinemic clamp.
Effect of starvation and diabetes on the sensitivity of carnitine palmitoyltransferase I to inhibition by 4-hydroxyphenylglyoxylate.
Effect of starvation on hepatic acyl-CoA synthetase, carnitine palmitoyltransferase-I, and acetyl-CoA carboxylase mRNA levels in rats.
Effects of Starvation on Lipid Metabolism and Gluconeogenesis in Yak.
Effects of starvation on the carnitine palmitoyltransferase of hepatic peroxisomes.
Effects of thyroidectomy and starvation on the activity and properties of hepatic carnitine palmitoyltransferase.
Exercise attenuates the fasting-induced transcriptional activation of metabolic genes in skeletal muscle.
Hepatic mitochondrial and peroxisomal oxidative capacity in riboflavin deficiency: effect of age, dietary fat and starvation in rats.
Hepatic mitochondrial function in ketogenic states. Diabetes, starvation, and after growth hormone administration.
Hepatic mitochondrial function in lean and obese Zucker rats.
Hepatic mitochondrial inner membrane properties and carnitine palmitoyltransferase A and B. Effect of diabetes and starvation.
Hepatic mitochondrial inner-membrane properties, beta-oxidation and carnitine palmitoyltransferases A and B. Effects of genetic obesity and starvation.
Insulin regulates enzyme activity, malonyl-CoA sensitivity and mRNA abundance of hepatic carnitine palmitoyltransferase-I.
Rat liver carnitine palmitoyltransferase 1 forms an oligomeric complex within the outer mitochondrial membrane.
Rat liver mitochondrial carnitine palmitoyltransferase-I, hepatic carnitine, and malonyl-CoA: effect of starvation.
Regulation of carnitine palmitoyltransferase activity in the liver and brown adipose tissue in the newborn rat: effect of starvation and hypothermia.
Regulation of carnitine palmitoyltransferase in vivo by glucagon and insulin.
Response to starvation of hepatic carnitine palmitoyltransferase activity and its regulation by malonyl-CoA. Sex differences and effects of pregnancy.
Some aspects of fatty acid oxidation in isolated fat-cell mitochondria from rat.
The effect of malonyl-CoA on overt and latent carnitine acyltransferase activities in rat liver and adipocyte mitochondria.
The effect of starvation on obese mice.
The role of changes in the sensitivity of hepatic mitochondrial overt carnitine palmitoyltransferase in determining the onset of the ketosis of starvation in the rat.
Use of a selectively permeabilized isolated rat hepatocyte preparation to study changes in the properties of overt carnitine palmitoyltransferase activity in situ.
Status Epilepticus
A Case of Recurrent Acute Encephalopathy with Febrile Convulsive Status Epilepticus with Carnitine Palmitoyltransferase II Variation.
Atypical presentation of carnitine palmitoyltransferase (CPT) deficiency as status epilepticus.
Stomach Neoplasms
Enhanced fatty acid oxidation mediated by CPT1C promotes gastric cancer progression.
Lipogenic gene expression profile in patients with gastric cancer.
Teratocarcinoma
NR4A nuclear receptors mediate carnitine palmitoyltransferase 1A gene expression by the rexinoid HX600.
Thyroid Cancer, Papillary
Cpt1c regulated by AMPK promotes papillary thyroid carcinomas cells survival under metabolic stress conditions.
Thyroid Carcinoma, Anaplastic
Growth inhibition of orthotopic anaplastic thyroid carcinoma xenografts in nude mice by PTK787/ZK222584 and CPT-11.
Triple Negative Breast Neoplasms
Dual inhibition of glutaminase and carnitine palmitoyltransferase decreases growth and migration of glutaminase inhibition-resistant triple-negative breast cancer cells.
Tyrosinemias
Targeted metabolomic analysis of plasma samples for the diagnosis of inherited metabolic disorders.
Urinary Bladder Neoplasms
Human and rat tumour cells possess mitochondrial carnitine palmitoyltransferase I and II: effects of insulin.
Multi-omics Integration Analysis Robustly Predicts High-Grade Patient Survival and Identifies CPT1B Effect on Fatty Acid Metabolism in Bladder Cancer.
Vitamin B 12 Deficiency
Vitamin B12 and omega-3 fatty acids together regulate lipid metabolism in Wistar rats.
Vitamin D Deficiency
Vitamin D Deficiency Attenuates Acute Alcohol-Induced Hepatic Lipid Accumulation in Mice.
Zellweger Syndrome
Lethal neonatal deficiency of carnitine palmitoyltransferase II associated with dysgenesis of the brain and kidneys.