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Disease on EC 3.7.1.2 - fumarylacetoacetase

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Acidosis
Differential impacts of elevated CO2 and acidosis on the energy budget of gill and liver cells from Atlantic cod, Gadus morhua.
Acute Kidney Injury
Sub-nephrotoxic cisplatin sensitizes rats to acute renal failure and increases urinary excretion of fumarylacetoacetase.
Adenocarcinoma
Activity of flavone acetic acid (NSC-347512) against solid tumors of mice.
Flavone acetic acid (LM-975; NSC-347512) activation to cytotoxic species in vivo and in vitro.
Flavone acetic acid antitumour activity against a mouse pancreatic adenocarcinoma is mediated by natural killer cells.
Influence of site on the chemosensitivity of transplantable murine colon tumours to flavone acetic acid (LM975, NSC 347512).
The ability of coumarin-, flavanon- and flavonol-analogues of flavone acetic acid to stimulate human monocytes.
Adenoma
[Immunohistochemical study in differential diagnosis of benign and malignant lesions of the thyroid gland]
Alkaptonuria
In vivo suppressor mutations correct a murine model of hereditary tyrosinemia type I.
Alzheimer Disease
Alzheimer's disease biomarkers: another tool for FAA pilot screening?
Anemia
Folate status and folate related anemia: a comparative cross-sectional study of long-term care and post-acute care psychogeriatric patients.
Aneurysm
Clinical relationship between femoral artery aneurysms and arteriomegaly.
Exacerbation of immunoglobulin G4-related inflammatory abdominal aortic aneurysm after endovascular repair.
Identification of a chromosome 11q23.2-q24 locus for familial aortic aneurysm disease, a genetically heterogeneous disorder.
Recurrent femoral anastomotic aneurysms. A 30-year experience.
[Anastomotic pseudoaneurysm, true para-anastomotic aneurysm and recurrent aneurysm following surgery for abdominal aortic aneurysm. Is a unifying theory possible?]
Anhedonia
EEG marker of inhibitory brain activity correlates with resting-state cerebral blood flow in the reward system in major depression.
Emotion self-regulation training in major depressive disorder using simultaneous real-time fMRI and EEG neurofeedback.
Arthritis, Experimental
Inverse relationship of hippocampal serotonin to avoidance behavior, serotonergic activation by emotional stress differentiated by estrous cycle and surgical stress.
Asthma
The Montelukast Therapy in Asthmatic Children with and without Food Allergy: Does It Make Any Difference?
Atrial Fibrillation
Electrophysiologic properties of atrial muscle in paroxysmal atrial fibrillation.
Bone Resorption
Skeletal lipidomics: regulation of bone metabolism by fatty acid amide family.
Brain Death
Does brain death induce a pro-inflammatory response at the organ level in a porcine model?
Brain Injuries
Attenuation of brain free fatty acid liberation during global ischemia: a model for screening potential therapies for efficacy?
Brain Ischemia
The influence of MK-801 on the hippocampal free arachidonic acid level and Na+,K+-ATPase activity in global cerebral ischemia-exposed rats.
Breast Neoplasms
Mutation analysis of the Fanconi anaemia A gene in breast tumours with loss of heterozygosity at 16q24.3.
Nocturnal 5-fluorouracil infusion to patients with breast cancer prior to surgery: appearance of 5-fluorouracil-induced AgNORs aggregation (FAA).
Carcinogenesis
A new nucleosomal protein in normal liver related to the cytoplasmic polypeptide target of a carcinogen.
Carcinogenicity of betel quid. III. Enhancement of 4-nitroquinoline-1-oxide- and N-2-fluorenylacetamide-induced carcinogenesis in rats by subsequent administration of betel nut.
Dose-dependent reduction of N-2-fluorenylacetamide-induced liver cancer and enhancement of bladder cancer in rats by butylated hydroxytoluene.
Late target protein of the carcinogen N-2-fluorenylacetamide in rat liver.
Mitosis in hepatocytes is generally associated with elevated levels of the target polypeptide of a liver carcinogen.
Point mutation instability (PIN) mutator phenotype as model for true back mutations seen in hereditary tyrosinemia type 1 - a hypothesis.
Carcinoma
Combined treatments of heat, radiation, or cytokines with flavone acetic acid on the growth of cultured endothelial cells.
Differential efficacy of flavone acetic against liver versus lung metastases in a human tumour xenograft.
Enhancement of in vitro cytotoxicity of mouse peritoneal exudate cells by flavone acetic acid (NSC 347512).
Enhancement of the antitumor effect of flavone acetic acid by the bioreductive cytotoxic drug SR 4233 in a murine carcinoma.
[Immunohistochemical study in differential diagnosis of benign and malignant lesions of the thyroid gland]
Carcinoma, Hepatocellular
Effects of carbon tetrachloride and azathioprine on diethylnitrosamine and N-2-fluorenylacetamide-induced hyperplastic liver nodule and hepatocellular carcinoma.
Efficient production of Fah-null heterozygote pigs by chimeric adeno-associated virus-mediated gene knockout and somatic cell nuclear transfer.
Enzyme deviation patterns in primary rat hepatomas induced by sequential administration of two chemically different carcinogens.
Renal function in tyrosinaemia type I after liver transplantation: A long-term follow-up.
Carcinoma, Lewis Lung
Enhancement of in vitro cytotoxicity of mouse peritoneal exudate cells by flavone acetic acid (NSC 347512).
Carcinoma, Papillary
[Immunohistochemical study in differential diagnosis of benign and malignant lesions of the thyroid gland]
Carcinoma, Squamous Cell
5,6-dimethylxanthenone-4-acetic acid (DMXAA): a new biological response modifier for cancer therapy.
Combined treatments of heat, radiation, or cytokines with flavone acetic acid on the growth of cultured endothelial cells.
Colonic Neoplasms
Activity of flavone acetic acid (NSC-347512) against solid tumors of mice.
Combination of flavone acetic acid (FAA) with adriamycin, cis-platinum and difluoromethylornithine (DFMO) in vitro against human colon cancer cells.
Flavone acetic acid (LM-975; NSC-347512) activation to cytotoxic species in vivo and in vitro.
Flavone acetic acid suppresses human peripheral blood lymphocyte and human colonic lamina propria lymphocyte DNA synthesis.
The activity of flavone acetic acid (NSC 347512) on human colon cancer cells in vitro.
Colorectal Neoplasms
Response to flavone acetic acid (NSC 347512) of primary and metastatic human colorectal carcinoma xenografts.
Compartment Syndromes
Treatment with covered stent of giant femoral artery aneurysm causing deep vein thrombosis.
Congenital Abnormalities
Single Cut Distal Femoral Varus Osteotomy (SCFO): A Preliminary Study.
Contracture
Isoforce: A new outrigger system for static progressive orthotic interventions of the proximal interphalangeal joint with constant force transmission-Results of a biomechanical study.
Coronary Artery Disease
A comparison of postmortem coronary atherosclerosis findings in general aviation pilot fatalities.
COVID-19
Changes in Social Media Impact of the Radiological Literature During the Covid-19 Pandemic.
Cysts
Diaphragmatic mesothelial cyst in a child with Fanconi aplastic anemia.
Echinococcus granulosus: a comparison of free amino acid concentration in hydatid fluid from primary and secondary cysts and host plasma.
Effect of mebendazole on free amino acid composition of cyst wall and cyst fluid of Echinococcus granulosus harbored in mice.
Deafness
Hearing deficit in a birth cohort of U.S. male commuter air carrier and air taxi pilots.
Dehydration
Effects of fixation and dehydration procedures on marine nematodes.
Exogenous ?-aminobutyric acid (GABA)-induced signaling events and field performance associated with mitigation of drought stress in Phaseolus vulgaris L.
The complex response of free and bound amino acids to water stress during the seed setting stage in Arabidopsis.
Dermatitis, Atopic
Quantitative Analysis of Free Amino Acids and Urea Derived from Isolated Corneocytes of Healthy Young, Healthy Aged, and Diseased Skin.
Diabetes Mellitus
Femoral anastomotic aneurysms: a continuing challenge.
Dystonia
Dystonia, botulinum neurotoxin, and the aviator.
Eczema
Antioxidant capacity of flavonoids from Folium Artemisiae Argyi and the molecular mechanism in Caenorhabditis elegans.
Fanconi Anemia
Expression cloning of a cDNA for the major Fanconi anaemia gene, FAA.
Functional activity of the fanconi anemia protein FAA requires FAC binding and nuclear localization.
Identification of Alu-mediated deletions in the Fanconi anemia gene FAA.
Normal expression of the Fanconi anemia proteins FAA and FAC and sensitivity to mitomycin C in two patients with Seckel syndrome.
Sequence variation in the Fanconi anemia gene FAA.
The Fanconi anaemia proteins, FAA and FAC, interact to form a nuclear complex.
The fanconi anemia pathway requires FAA phosphorylation and FAA/FAC nuclear accumulation.
The Fanconi anemia proteins FAA and FAC function in different cellular compartments to protect against cross-linking agent cytotoxicity.
Fibromyalgia
Free amino acids in fibromyalgia syndrome: relationship with clinical picture.
Fibrosarcoma
Effect of environmental conditions (pH, oxygenation and temperature) on the cytotoxicity of flavone acetic acid and its dimethylaminoethyl ester.
Flavone acetic acid as a modifier of endothelial cell function.
fumarylacetoacetase deficiency
A novel mutation causing mild, atypical fumarylacetoacetase deficiency (Tyrosinemia type I): a case report.
Biochemical studies of a patient with hereditary hepatorenal tyrosinemia: evidence of glutathione deficiency.
Cell fusion is the principal source of bone-marrow-derived hepatocytes.
Characterization of a fungal maleylacetoacetate isomerase gene and identification of its human homologue.
Correction of fumarylacetoacetate hydrolase deficiency (type I tyrosinemia) in cultured human fibroblasts by retroviral-mediated gene transfer.
Different molecular basis for fumarylacetoacetate hydrolase deficiency in the two clinical forms of hereditary tyrosinemia (type I).
Effect of nitisinone (NTBC) treatment on the clinical course of hepatorenal tyrosinemia in Québec.
Fumarylacetoacetate, the metabolite accumulating in hereditary tyrosinemia, activates the ERK pathway and induces mitotic abnormalities and genomic instability.
Induced pluripotent stem cell-derived hepatocytes have the functional and proliferative capabilities needed for liver regeneration in mice.
Infants with Tyrosinemia Type 1: Should phenylalanine be supplemented?
Murine fumarylacetoacetate hydrolase (Fah) gene is disrupted by a neonatally lethal albino deletion that defines the hepatocyte-specific developmental regulation 1 (hsdr-1) locus.
Serial transplantation reveals the stem-cell-like regenerative potential of adult mouse hepatocytes.
Succinylacetone as primary marker to detect tyrosinemia type I in newborns and its measurement by newborn screening programs.
Tandem mass spectrometric determination of succinylacetone in dried blood spots enables presymptomatic detection in a case of hepatorenal tyrosinaemia.
The nephropathy of type I tyrosinemia after liver transplantation.
The pathophysiology and treatment of hereditary tyrosinemia type 1.
The pre- and post-natal diagnosis of tyrosinemia type I and the detection of the carrier state by assay of fumarylacetoacetase.
The Unique Spectrum of Mutations in Patients with Hereditary Tyrosinemia Type 1 in Different Regions of the Russian Federation.
Tyrosinaemia--treatment and outcome.
Genetic Diseases, Inborn
A GC/MS validated method for the nanomolar range determination of succinylacetone in amniotic fluid and plasma: An analytical tool for tyrosinemia type I.
Animal models reveal pathophysiologies of tyrosinemias.
Cas9-nickase-mediated genome editing corrects hereditary tyrosinemia in rats.
Gene expression profiles of homogentisate-treated Fah-/- Hpd-/-mice using DNA microarrays.
Imaging features of type 1 hereditary tyrosinemia: a review of 30 patients.
Murine fumarylacetoacetate hydrolase (Fah) gene is disrupted by a neonatally lethal albino deletion that defines the hepatocyte-specific developmental regulation 1 (hsdr-1) locus.
Nitisinone in the treatment of hereditary tyrosinaemia type 1.
Point mutations in the murine fumarylacetoacetate hydrolase gene: Animal models for the human genetic disorder hereditary tyrosinemia type 1.
Tyrosinemia: the Quebec experience.
Glaucoma
Glaucoma in U.S. Civil Aviation: 2005-2014.
Glioma
Deregulation of ion channel and transporter encoding genes in pediatric gliomas.
Glucose Intolerance
Unravelling the mysterious roles of melanocortin-3 receptors in metabolic homeostasis and obesity using mouse genetics.
Goiter, Nodular
[Immunohistochemical study in differential diagnosis of benign and malignant lesions of the thyroid gland]
Graves Disease
Evidence for anti-tubulin autoantibodies in the form of immune complexes in human sera.
Hashimoto Disease
[Immunohistochemical study in differential diagnosis of benign and malignant lesions of the thyroid gland]
Heart Diseases
Recurrent femoral anastomotic aneurysms. A 30-year experience.
Heart Failure
[False anastomotic aneurysms after aorto-femoral prosthesis. Detection, prevention and treatment]
Hepatitis
Autoimmune hepatitis-like disease in C57BL/6 mice infected with mouse hepatitis virus A59.
Detection of mouse hepatitis virus infection by assay of anti-liver autoantibodies.
Effects of interleukin 17A (IL-17A) neutralization on murine hepatitis virus (MHV-A59) infection.
Fine specificity of autoantibodies induced by mouse hepatitis virus A59.
Levo-1-methyl tryptophan aggravates the effects of mouse hepatitis virus (MHV-A59) infection.
Prevalence of IgG autoantibody against F-actin in patients suspected of having autoimmune or acute viral hepatitis.
Sequence similarity and structural homologies are involved in the autoimmune response elicited by mouse hepatitis virus A59.
The peptide specificities of the autoantibodies elicited by mouse hepatitis virus A59.
Uric acid and HMGB1 are involved in the induction of autoantibodies elicited in mice infected with mouse hepatitis virus A59.
Hepatitis B
Modeling hepatitis B virus X-induced hepatocellular carcinoma in mice with the sleeping beauty transposon system.
Studying HBV Infection and Therapy in Immune-Deficient NOD-Rag1-/-IL2RgammaC-null (NRG) Fumarylacetoacetate Hydrolase (Fah) Knockout Mice Transplanted with Human Hepatocytes.
Hypersensitivity
Fumarylacetoacetate hydrolase is involved in salt stress response in Arabidopsis.
Hypotension
Flavone acetic acid (FAA) with recombinant interleukin-2 (rIL-2) in advanced malignant melanoma: I. Clinical and vascular studies.
Hypoxia, Brain
The influence of nimodipine and MK-801 on the brain free arachidonic acid level and the learning ability in hypoxia-exposed rats.
Immunoglobulin G4-Related Disease
Exacerbation of immunoglobulin G4-related inflammatory abdominal aortic aneurysm after endovascular repair.
Infections
Effects of starvation and infection with Schistosoma mansoni on the release rate of free amino acids (FAA) by Biomphalaria glabrata.
Studying HBV Infection and Therapy in Immune-Deficient NOD-Rag1-/-IL2RgammaC-null (NRG) Fumarylacetoacetate Hydrolase (Fah) Knockout Mice Transplanted with Human Hepatocytes.
Insulin Resistance
Unravelling the mysterious roles of melanocortin-3 receptors in metabolic homeostasis and obesity using mouse genetics.
Interatrial Block
Electrophysiologic properties of atrial muscle in paroxysmal atrial fibrillation.
Iron Deficiencies
Acute telogen effluvium onset event is associated with the presence of female androgenetic alopecia: potential therapeutic implications.
Iron Overload
Deferasirox therapy in children with Fanconi aplastic anemia.
Ischemic Attack, Transient
Recurrent Stroke Risk in Pilots with Atrial Fibrillation.
Kidney Calculi
The amino acid factor in stone formers' and normal urines.
Kidney Failure, Chronic
Plasma free amino acids and their metabolites in Taiwanese patients on hemodialysis and continuous ambulatory peritoneal dialysis.
Kidney Neoplasms
Augmentation of natural killer activity, induction of IFN and development tumor immunity during the successful treatment of established murine renal cancer using flavone acetic acid and IL-2.
Correlation between in vivo induction of cytokine gene expression by flavone acetic acid and strict dose dependency and therapeutic efficacy against murine renal cancer.
Flavone 8-acetic acid: our current understanding of its mechanism of action in solid tumours.
Flavone-8-acetic acid augments systemic natural killer cell activity and synergizes with IL-2 for treatment of murine renal cancer.
Systemic alkalinization inhibits the ability of flavone acetic acid to augment natural killer activity, induce cytokine gene expression, and synergize with interleukin 2 for the treatment of murine renal cancer.
Leukemia
Activity of flavone acetic acid (NSC-347512) against solid tumors of mice.
Effect of flavone acetic acid on Lewis lung carcinoma: evidence for an indirect effect.
Interaction between flavone acetic acid (LM-975, NSC 349512) and radiation in Glasgow's osteogenic sarcoma in vivo.
Liver Diseases
Aneuploidy as a mechanism for stress-induced liver adaptation.
Cell fusion is the principal source of bone-marrow-derived hepatocytes.
Ex Vivo Cell Therapy by Ectopic Hepatocyte Transplantation Treats the Porcine Tyrosinemia Model of Acute Liver Failure.
Fumarylacetoacetate hydrolase deficient pigs are a novel large animal model of metabolic liver disease.
Heat shock response associated with hepatocarcinogenesis in a murine model of hereditary tyrosinemia type I.
Hepatocytes corrected by gene therapy are selected in vivo in a murine model of hereditary tyrosinaemia type I.
Myelomonocytic cells are sufficient for therapeutic cell fusion in liver.
Succinylacetone as primary marker to detect tyrosinemia type I in newborns and its measurement by newborn screening programs.
Targeting the Apoa1 locus for liver-directed gene therapy.
Liver Failure
Brief report: Parthenogenetic embryonic stem cells are an effective cell source for therapeutic liver repopulation.
CRISPR/Cas9-mediated Targeted Integration In Vivo Using a Homology-mediated End Joining-based Strategy.
Dendrimer-Based Lipid Nanoparticles Deliver Therapeutic FAH mRNA to Normalize Liver Function and Extend Survival in a Mouse Model of Hepatorenal Tyrosinemia Type I.
Efficient production of Fah-null heterozygote pigs by chimeric adeno-associated virus-mediated gene knockout and somatic cell nuclear transfer.
Ex Vivo Hepatocyte Reprograming Promotes Homology-Directed DNA Repair to Correct Metabolic Disease in Mice After Transplantation.
Ex Vivo Cell Therapy by Ectopic Hepatocyte Transplantation Treats the Porcine Tyrosinemia Model of Acute Liver Failure.
Infiltrating neutrophils aggravate metabolic liver failure in fah-deficient mice.
Noninvasive 3D imaging of liver regeneration in a mouse model of hereditary tyrosinemia type 1 using the sodium iodide symporter gene.
Renal function in tyrosinaemia type I after liver transplantation: A long-term follow-up.
Liver Neoplasms
Abnormalities in liver iron accumulation during N-2-fluorenylacetamide hepatocarcinogenesis that are dependent or independent of continued carcinogen action.
Absence of a promoting or sequential syncarcinogenic effect in rat liver by the carcinogenic hypolipidemic drug nafenopin given after N-2-fluorenylacetamide.
An enhancing effect of the antihistaminic drug methapyrilene on rat liver carcinogenesis by previously administered N-2-fluorenylacetamide.
Dose-dependent reduction of N-2-fluorenylacetamide-induced liver cancer and enhancement of bladder cancer in rats by butylated hydroxytoluene.
Fumarylacetoacetate, the metabolite accumulating in hereditary tyrosinemia, activates the ERK pathway and induces mitotic abnormalities and genomic instability.
Lack of rapid initiating, promoting or sequential syncarcinogenic effects of di(2-ethylhexyl)phthalate in rat liver carcinogenesis.
Lung Diseases
Prevalence of feet and ankle arthritis and their impact on clinical indices in patients with rheumatoid arthritis: a cross-sectional study.
Lung Neoplasms
Feruloyl-L-arabinose attenuates migration, invasion and production of reactive oxygen species in H1299 lung cancer cells.
Lymphoma
Enhancement of in vitro cytotoxicity of mouse peritoneal exudate cells by flavone acetic acid (NSC 347512).
Induction of natural killer cell activity by the antitumour compound flavone acetic acid (NSC 347 512).
Lymphoma, Non-Hodgkin
Activity of flavone acetic acid (NSC-347512) against solid tumors of mice.
Mastocytoma
Enhancement of in vitro cytotoxicity of mouse peritoneal exudate cells by flavone acetic acid (NSC 347512).
Melanoma
CDC5L drives FAH expression to promote metabolic reprogramming in melanoma.
Flavone acetic acid (FAA) with recombinant interleukin-2 (rIL-2) in advanced malignant melanoma: I. Clinical and vascular studies.
Flavone acetic acid induced changes in human endothelial permeability: potentiation by tumour-conditioned medium.
Tumour concentrations of flavone acetic acid (FAA) in human melanoma: comparison with mouse data.
Melanoma, Experimental
Flavone acetic acid increases the antitumor effect of hyperthermia in mice.
Flavone acetic acid increases the cytotoxicity of mitomycin C when combined with hyperthermia.
In vivo effects of tumor necrosis factor-alpha or flavone acetic acid in combination with doxorubicin on multidrug-resistant B16 melanoma.
Vasoacting agents flavone acetic acid and hydralazine given in combination enhance antitumor effects under condition of hyperthermia.
Metabolic Diseases
A Case Report of a Very Rare Association of Tyrosinemia type I and Pancreatitis Mimicking Neurologic Crisis of Tyrosinemia Type I.
Cytoplasmic nonsense-mediated mRNA decay for a nonsense (W262X) transcript of the gene responsible for hereditary tyrosinemia, fumarylacetoacetate hydrolase.
Experience of a Single Center in NTBC Use in Management of Hereditary Tyrosinemia Type I in Libya.
Fumarylacetoacetate, the metabolite accumulating in hereditary tyrosinemia, activates the ERK pathway and induces mitotic abnormalities and genomic instability.
Identification of a stop mutation in five Finnish patients suffering from hereditary tyrosinemia type I.
Pharmacological correction of neonatal lethal hepatic dysfunction in a murine model of hereditary tyrosinaemia type I.
Point mutations in the murine fumarylacetoacetate hydrolase gene: Animal models for the human genetic disorder hereditary tyrosinemia type 1.
Revisiting hereditary tyrosinemia Type 1-spectrum of radiological findings.
Spectrum of mutations in the fumarylacetoacetate hydrolase gene of tyrosinemia type 1 patients in northwestern Europe and Mediterranean countries.
The mutagenicity of the tyrosine metabolite, fumarylacetoacetate, is enhanced by glutathione depletion.
Tyrosinemia type 1 and irreversible neurologic crisis after one month discontinuation of nitisone.
Neoplasm Metastasis
Augmentation of natural killer activity, induction of IFN and development tumor immunity during the successful treatment of established murine renal cancer using flavone acetic acid and IL-2.
Response of primary tumour, spontaneous metastases and recurrence of Lewis lung carcinoma (3LL) to flavone acetic acid (FAA, LM975).
Response to flavone acetic acid (NSC 347512) of primary and metastatic human colorectal carcinoma xenografts.
Neoplasm, Residual
Augmentation of natural killer activity, induction of IFN and development tumor immunity during the successful treatment of established murine renal cancer using flavone acetic acid and IL-2.
Neoplasms
5,6-dimethylxanthenone-4-acetic acid (DMXAA): a new biological response modifier for cancer therapy.
Absence of a promoting or sequential syncarcinogenic effect in rat liver by the carcinogenic hypolipidemic drug nafenopin given after N-2-fluorenylacetamide.
Activity of flavone acetic acid (NSC-347512) against solid tumors of mice.
Acute effects of vascular modifying agents in solid tumors assessed by noninvasive laser Doppler flowmetry and near infrared spectroscopy.
Anti-tumour activity of flavone acetic acid (NSC 347512) in mice--influence of immune status.
Anti-vascular approaches to solid tumour therapy: evaluation of vinblastine and flavone acetic acid.
Anticancer Flavonoids Are Mouse-Selective STING Agonists.
Antitumor activity of chemical modified natural compounds.
Antitumor effects of alpha-interferon and gamma-interferon on a murine renal cancer (Renca) in vitro and in vivo.
Antivascular approaches to solid tumour therapy: evaluation of tubulin binding agents.
Augmentation of natural killer activity, induction of IFN and development tumor immunity during the successful treatment of established murine renal cancer using flavone acetic acid and IL-2.
Blood flow failure as a major determinant in the antitumor action of flavone acetic acid.
Changes in coagulation and permeability properties of human endothelial cells in vitro induced by TNF-alpha or 5,6 MeXAA.
Combined treatments of heat, radiation, or cytokines with flavone acetic acid on the growth of cultured endothelial cells.
Contrast-enhanced ultrasound-guided feeding artery ablation as add-on to percutaneous radiofrequency ablation for hypervascular hepatocellular carcinoma with a modified ablative technique and tumor perfusion evaluation.
Correlation between immune and vascular activities of xanthenone acetic acid antitumor agents.
Correlation between in vivo induction of cytokine gene expression by flavone acetic acid and strict dose dependency and therapeutic efficacy against murine renal cancer.
Dendrimer-Based Lipid Nanoparticles Deliver Therapeutic FAH mRNA to Normalize Liver Function and Extend Survival in a Mouse Model of Hepatorenal Tyrosinemia Type I.
Differential efficacy of flavone acetic against liver versus lung metastases in a human tumour xenograft.
Divergent effects of flavone acetic acid on established versus developing tumour blood flow.
Effect of environmental conditions (pH, oxygenation and temperature) on the cytotoxicity of flavone acetic acid and its dimethylaminoethyl ester.
Effect of flavone acetic acid (NSC 347,512) on splenic cytotoxic effector cells and their role in tumour necrosis.
Effect of flavone acetic acid on endothelial cell proliferation: evidence for antiangiogenic properties.
Effect of flavone acetic acid on Lewis lung carcinoma: evidence for an indirect effect.
Effects of boron neutron capture therapy using borocaptate sodium in combination with a tumor-selective vasoactive agent in mice.
Eltrombopag Add-on Treatment in a Child With Fanconi Aplastic Anemia Awaiting Hematopoietic Stem Cell Transplantation.
Enhancement of hepatocarcinogenesis by sequential administration of chemicals: summation versus promotion effects.
Enhancement of in vitro cytotoxicity of mouse peritoneal exudate cells by flavone acetic acid (NSC 347512).
Enhancement of mitomycin-C cytotoxicity by combination with flavone acetic acid in a murine tumour.
Enhancement of radioimmunotherapy by drugs modifying tumour blood flow in a colonic xenograft model.
Enhancement of the antitumor effect of flavone acetic acid by the bioreductive cytotoxic drug SR 4233 in a murine carcinoma.
Evidence for the production of nitric oxide by activated macrophages treated with the antitumor agents flavone-8-acetic acid and xanthenone-4-acetic acid.
Feruloyl-L-arabinose attenuates migration, invasion and production of reactive oxygen species in H1299 lung cancer cells.
Flavone 8-acetic acid: our current understanding of its mechanism of action in solid tumours.
Flavone acetic acid (FAA) with recombinant interleukin-2 (rIL-2) in advanced malignant melanoma. IV: Pharmacokinetics and toxicity of flavone acetic acid and its metabolites.
Flavone acetic acid (FAA) with recombinant interleukin-2 (rIL-2) in advanced malignant melanoma: I. Clinical and vascular studies.
Flavone acetic acid (LM-975; NSC-347512) activation to cytotoxic species in vivo and in vitro.
Flavone acetic acid (NSC 347512)-induced modulation of murine tumor physiology monitored by in vivo nuclear magnetic resonance spectroscopy.
Flavone acetic acid and plasma protein binding.
Flavone acetic acid antitumour activity against a mouse pancreatic adenocarcinoma is mediated by natural killer cells.
Flavone acetic acid as a modifier of endothelial cell function.
Flavone acetic acid directly induces expression of cytokine genes in mouse splenic leukocytes but not in human peripheral blood leukocytes.
Flavone acetic acid distribution in human malignant tumors.
Flavone acetic acid increases the antitumor effect of hyperthermia in mice.
Flavone acetic acid increases the cytotoxicity of mitomycin C when combined with hyperthermia.
Flavone acetic acid induced changes in human endothelial permeability: potentiation by tumour-conditioned medium.
Flavone acetic acid induces a coagulopathy in mice.
Flavone acetic acid pharmacokinetics in nude mice.
Flavone acetic acid potentiates the induction of endothelial procoagulant activity by tumour necrosis factor.
Flavone acetic acid stimulates nitric oxide and peroxynitrite production in subcutaneous mouse tumors.
Flavone-8-acetic acid augments systemic natural killer cell activity and synergizes with IL-2 for treatment of murine renal cancer.
Hepatocellular carcinoma despite long-term survival in chronic tyrosinaemia I.
High volume naked DNA tail-vein injection restores liver function in Fah-knock out mice.
Immunological effects of flavone acetic acid.
Inadequate vasculature in solid tumours: consequences for cancer research strategies.
Induction of hypoxia in the KHT sarcoma by tumour necrosis factor and flavone acetic acid.
Induction of multiple cytokine gene expression and IRF-1 mRNA by flavone acetic acid in a murine macrophage cell line.
Induction of natural killer activity by xanthenone analogues of flavone acetic acid: relation with antitumour activity.
Induction of natural killer cell activity by the antitumour compound flavone acetic acid (NSC 347 512).
Induction of tumour hypoxia by FAA and TNF: interaction with bioreductive drugs.
Influence of site on the chemosensitivity of transplantable murine colon tumours to flavone acetic acid (LM975, NSC 347512).
Inhibition of antitumor effects of flavone acetic acid by cortisone.
Inhibitory effect of ellagic acid on N-2-fluorenylacetamide-induced liver carcinogenesis in male ACI/N rats.
Interaction between flavone acetic acid (LM-975, NSC 349512) and radiation in Glasgow's osteogenic sarcoma in vivo.
Long-term effects of flavone acetic acid on the growth of a rat tumour.
Mechanisms of the inhibitory action of p-hydroxyacetanilide on carcinogenesis by N-2-fluorenylacetamide or N-hydroxy-N-2-fluorenylacetamide.
Nitric oxide: its production in host-cell-infiltrated EMT6 spheroids and its role in tumour cell killing by flavone-8-acetic acid and 5,6-dimethylxanthenone-4-acetic acid.
Nocturnal 5-fluorouracil infusion to patients with breast cancer prior to surgery: appearance of 5-fluorouracil-induced AgNORs aggregation (FAA).
Nucleolar organizer regions in hepatocarcinogenesis induced by N-2-fluorenylacetamide in rats: comparison with bromodeoxyuridine immunohistochemistry.
Oxidative stress contributes to the anti-proliferative effects of flavone acetic acid on endothelial cells.
Percutaneous radiofrequency ablation of tumor feeding artery before target tumor ablation may reduce local tumor progression in hepatocellular carcinoma.
Pharmacodynamics and causes of dose-dependent pharmacokinetics of flavone-8-acetic acid (LM-975; NSC-347512) in mice.
Potentiation of the cytotoxicity of carboquone by flavone acetic acid combined with hyperthermia.
Preclinical in vitro and in vivo activity of 5,6-dimethylxanthenone-4-acetic acid.
Production of tumour necrosis factor-alpha by cultured human peripheral blood leucocytes in response to the anti-tumour agent 5,6-dimethylxanthenone-4-acetic acid (NSC 640488).
Recombinant interleukin-2 (rIL-2) with flavone acetic acid (FAA) in advanced malignant melanoma: a phase II study.
Reduction of cytotoxic effector cell activity in colon 38 tumours following treatment with flavone acetic acid.
Reduction of tumor blood flow by flavone acetic acid: a possible component of therapy.
Response of chemically induced primary colon tumours of the mouse to flavone acetic acid (NSC 347 512).
Response of primary tumour, spontaneous metastases and recurrence of Lewis lung carcinoma (3LL) to flavone acetic acid (FAA, LM975).
Response to flavone acetic acid (NSC 347512) of primary and metastatic human colorectal carcinoma xenografts.
Responses of experimental rat tumours and a mouse colon tumour to flavone acetic acid.
Review article: angiogenesis, neovascular proliferation and vascular pathophysiology as targets for cancer therapy.
Role of T cells and tumour necrosis factor in antitumour activity and toxicity of flavone acetic acid.
Role of tumor necrosis factor in flavone acetic acid-induced tumor vasculature shutdown.
Selective induction of endothelial cell tissue factor in the presence of a tumour-derived mediator: a potential mechanism of flavone acetic acid action in tumour vasculature.
Serotonin involvement in the antitumour and host effects of flavone-8-acetic acid and 5,6-dimethylxanthenone-4-acetic acid.
Small-molecule cytokine inducers causing tumor necrosis.
Studies with a panel of tumours having a variable sensitivity to FAA, to investigate its mechanism of action.
Synthesis and antitumour activity of new derivatives of flavone-8-acetic acid (FAA). Part 1: 6-Methyl derivatives.
Systemic alkalinization inhibits the ability of flavone acetic acid to augment natural killer activity, induce cytokine gene expression, and synergize with interleukin 2 for the treatment of murine renal cancer.
Systemic effects of FAA are enhanced by implanted tumours.
Testicular mesotheliomas in rats exposed to N-2-fluorenylacetamide (FAA).
The effect of 5,6-dimethylxanthenone-4-acetic acid on tumour necrosis factor production by human immune cells.
The effect of blood flow modification on intra- and extracellular pH measured by 31P magnetic resonance spectroscopy in murine tumours.
The in vivo interaction between flavone acetic acid and hyperthermia.
The relationship between tissue levels of flavone acetic acid (NSC 347512) and site dependent anti-tumour activity in murine colon tumours.
The role of immune effector cells in flavone acetic acid-induced injury to tumor cells in EMT6 spheroids.
The Sir Mark Oliphant Conferences: international frontiers of science and technology.
The use of vascularised spheroids to investigate the action of flavone acetic acid on tumour blood vessels.
Tumor vascular disrupting agent DMXAA inhibits platelet activation and thrombosis via inhibition of TXA2 signaling and phosphodiesterase.
Tumor-dependent increased plasma nitrate concentrations as an indication of the antitumor effect of flavone-8-acetic acid and analogues in mice.
Tumour concentrations of flavone acetic acid (FAA) in human melanoma: comparison with mouse data.
Unique chemosensitivity of MAC 16 tumours to flavone acetic acid (LM975, NSC 347512).
Vascular attack by 5,6-dimethylxanthenone-4-acetic acid combined with B7.1 (CD80)-mediated immunotherapy overcomes immune resistance and leads to the eradication of large tumors and multiple tumor foci.
Vascular collapse after flavone acetic acid: a possible mechanism of its anti-tumour action.
Vasoacting agents flavone acetic acid and hydralazine given in combination enhance antitumor effects under condition of hyperthermia.
Obesity
Co-morbid conditions in overweight and obese airmen: trends and aeromedical implications.
Food cues and ghrelin recruit the same neuronal circuitry.
GHS-R1a signaling in the DMH and VMH contributes to food anticipatory activity.
Unravelling the mysterious roles of melanocortin-3 receptors in metabolic homeostasis and obesity using mouse genetics.
Osteoporosis
Skeletal lipidomics: regulation of bone metabolism by fatty acid amide family.
Osteosarcoma
Flavone acetic acid (NSC 347512)-induced modulation of murine tumor physiology monitored by in vivo nuclear magnetic resonance spectroscopy.
Interaction between flavone acetic acid (LM-975, NSC 349512) and radiation in Glasgow's osteogenic sarcoma in vivo.
Overweight
Co-morbid conditions in overweight and obese airmen: trends and aeromedical implications.
Pancytopenia
Eltrombopag Add-on Treatment in a Child With Fanconi Aplastic Anemia Awaiting Hematopoietic Stem Cell Transplantation.
Patellar Dislocation
[Short-term effectiveness of derotational distal femoral osteotomy combined with medial patellofemoral ligament reconstruction for recurrent patellar dislocation].
Porphyria, Acute Intermittent
Renal function in tyrosinaemia type I after liver transplantation: A long-term follow-up.
Porphyrias
Renal function in tyrosinaemia type I after liver transplantation: A long-term follow-up.
[delta-Aminolevulinate dehydratase deficiency]
Porphyrias, Hepatic
[delta-Aminolevulinate dehydratase deficiency]
Pregnancy, High-Risk
Fumarylacetoacetase activity in cultured and non-cultured chorionic villus cells, and assay in two high-risk pregnancies.
Psoriasis
Quantitative Analysis of Free Amino Acids and Urea Derived from Isolated Corneocytes of Healthy Young, Healthy Aged, and Diseased Skin.
Pulmonary Heart Disease
Electrophysiological study of infant and adult rats under acute intoxication with fluoroacetamide.
Renal Insufficiency
[False anastomotic aneurysms after aorto-femoral prosthesis. Detection, prevention and treatment]
Rhabdomyosarcoma
Responses of experimental rat tumours and a mouse colon tumour to flavone acetic acid.
Sarcoma
Activity of flavone acetic acid (NSC-347512) against solid tumors of mice.
Induction of hypoxia in the KHT sarcoma by tumour necrosis factor and flavone acetic acid.
Long-term effects of flavone acetic acid on the growth of a rat tumour.
Seizures
Fatty Acid Amides Synthesized from Andiroba Oil (Carapa guianensis Aublet.) Exhibit Anticonvulsant Action with Modulation on GABA-A Receptor in Mice: A Putative Therapeutic Option.
Fractional anisotropy asymmetry and the side of seizure origin for partial onset-temporal lobe epilepsy.
Lessons learned from poisoning cases caused by 2 illegal rodenticides: Tetramine and fluoroacetamide.
Merging the structural motifs of functionalized amino acids and alpha-aminoamides: compounds with significant anticonvulsant activities.
Sepsis
[False anastomotic aneurysms after aorto-femoral prosthesis. Detection, prevention and treatment]
Sleep Deprivation
Sleep homeostasis during daytime food entrainment in mice.
Starvation
Effect of starvation on free histidine and amino acids in white muscle of milkfish Chanos chanos.
[A study on the constitutional change of taurine and other free amino acids during the fetal development (author's transl)]
Stroke
Clinical evaluation of a fully automated model-based algorithm to calculate left ventricular volumes and ejection fraction using multidetector computed tomography.
Recurrent Stroke Risk in Pilots with Atrial Fibrillation.
Testicular Neoplasms
Testicular tumor in an aviator.
Thrombosis
Flavone-8-acetic acid (Flavonoid) profoundly reduces platelet-dependent thrombosis and vasoconstriction after deep arterial injury In vivo.
Treatment with covered stent of giant femoral artery aneurysm causing deep vein thrombosis.
Tyrosinemias
A Case Report of a Very Rare Association of Tyrosinemia type I and Pancreatitis Mimicking Neurologic Crisis of Tyrosinemia Type I.
A Compensatory U1snRNA Partially Rescues FAH Splicing and Protein Expression in a Splicing-Defective Mouse Model of Tyrosinemia Type I.
A GC/MS validated method for the nanomolar range determination of succinylacetone in amniotic fluid and plasma: An analytical tool for tyrosinemia type I.
A missense mutation (Q279R) in the fumarylacetoacetate hydrolase gene, responsible for hereditary tyrosinemia, acts as a splicing mutation.
A mouse model of renal tubular injury of tyrosinemia type 1: development of de Toni Fanconi syndrome and apoptosis of renal tubular cells in Fah/Hpd double mutant mice.
A novel mutation causing mild, atypical fumarylacetoacetase deficiency (Tyrosinemia type I): a case report.
A single mutation of the fumarylacetoacetate hydrolase gene in French Canadians with hereditary tyrosinemia type I.
AAV Vectors Containing rDNA Homology Display Increased Chromosomal Integration and Transgene Persistence.
Abnormal social behavior in mice with tyrosinemia type I is associated with an increase of myelin in the cerebral cortex.
Adenovirus-mediated gene therapy in a mouse model of hereditary tyrosinemia type I.
Amelioration of an Inherited Metabolic Liver Disease through Creation of a De Novo Start Codon by Cytidine Base Editing.
Animal models reveal pathophysiologies of tyrosinemias.
Autologous Gene and Cell Therapy Provides Safe and Long-Term Curative Therapy in A Large Pig Model of Hereditary Tyrosinemia Type 1.
Biochemical studies on the enzymatic deficiencies in hereditary tyrosinemia.
Cas9-nickase-mediated genome editing corrects hereditary tyrosinemia in rats.
Characterization of a fungal maleylacetoacetate isomerase gene and identification of its human homologue.
Characterization of the human fumarylacetoacetate hydrolase gene and identification of a missense mutation abolishing enzymatic activity.
Chronic Phenotype Characterization of a Large-Animal Model of Hereditary Tyrosinemia Type 1.
Clinical and Biochemical Profile of Tyrosinemia Type 1 in Tunisia.
Clinical and para clinical findings in the children with tyrosinemia referring for liver transplantation.
Cloning and expression analysis of a cDNA encoding fumarylacetoacetate hydrolase: post-transcriptional modulation in rat liver and kidney.
Cloning and expression of the cDNA encoding human fumarylacetoacetate hydrolase, the enzyme deficient in hereditary tyrosinemia: assignment of the gene to chromosome 15.
Compound mutations (R237X and L375P) in the fumarylacetoacetate hydrolase gene causing tyrosinemia type I in a Chinese patient.
Correction of fumarylacetoacetate hydrolase deficiency (type I tyrosinemia) in cultured human fibroblasts by retroviral-mediated gene transfer.
Curative Ex Vivo Hepatocyte-Directed Gene Editing in a Mouse Model of Hereditary Tyrosinemia Type 1.
Current strategies for the treatment of hereditary tyrosinemia type I.
Cytoplasmic nonsense-mediated mRNA decay for a nonsense (W262X) transcript of the gene responsible for hereditary tyrosinemia, fumarylacetoacetate hydrolase.
Deficiency of fumarylacetoacetase in a patient with hereditary tyrosinemia.
Deficiency of fumarylacetoacetase without hereditary tyrosinemia.
Deficient DNA-ligase activity in the metabolic disease tyrosinemia type I.
Dendrimer-Based Lipid Nanoparticles Deliver Therapeutic FAH mRNA to Normalize Liver Function and Extend Survival in a Mouse Model of Hepatorenal Tyrosinemia Type I.
Diagnosis and management of tyrosinemia type I.
Different clinical forms of hereditary tyrosinemia (type I) in patients with identical genotypes.
Different molecular basis for fumarylacetoacetate hydrolase deficiency in the two clinical forms of hereditary tyrosinemia (type I).
DNA damage and repair in mammalian cells exposed to p-hydroxyphenylpyruvic acid.
Effect of nitisinone (NTBC) treatment on the clinical course of hepatorenal tyrosinemia in Québec.
Efficient liver repopulation of transplanted hepatocyte prevents cirrhosis in a rat model of hereditary tyrosinemia type I.
Efficient production of Fah-null heterozygote pigs by chimeric adeno-associated virus-mediated gene knockout and somatic cell nuclear transfer.
Evaluation of dichloroacetate treatment in a murine model of hereditary tyrosinemia type 1.
Experience of a Single Center in NTBC Use in Management of Hereditary Tyrosinemia Type I in Libya.
Expression and post-translational modification of human 4-hydroxy-phenylpyruvate dioxygenase.
Extensive changes in liver gene expression induced by hereditary tyrosinemia type I are not normalized by treatment with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC).
Ex Vivo Cell Therapy by Ectopic Hepatocyte Transplantation Treats the Porcine Tyrosinemia Model of Acute Liver Failure.
Four-hydroxyphenylpyruvic acid oxidase deficiency with normal fumarylacetoacetase: a new variant form of hereditary hypertyrosinemia.
Frequent mutation reversion inversely correlates with clinical severity in a genetic liver disease, hereditary tyrosinemia.
Fumarylacetoacetase measurement as a mass-screening procedure for hereditary tyrosinemia type I.
Fumarylacetoacetate hydrolase deficient pigs are a novel large animal model of metabolic liver disease.
Fumarylacetoacetate Hydrolase Knock-out Rabbit Model for Hereditary Tyrosinemia Type 1.
Fumarylacetoacetate, the metabolite accumulating in hereditary tyrosinemia, activates the ERK pathway and induces mitotic abnormalities and genomic instability.
Functional analysis and in vitro correction of splicing FAH mutations causing tyrosinemia type I.
Gene expression profiles of homogentisate-treated Fah-/- Hpd-/-mice using DNA microarrays.
Genetically blocking HPD via CRISPR-Cas9 protects against lethal liver injury in a pig model of tyrosinemia type I.
Geographical and Ethnic Distribution of Mutations of the Fumarylacetoacetate Hydrolase Gene in Hereditary Tyrosinemia Type 1.
Harnessing a high cargo-capacity transposon for genetic applications in vertebrates.
Heat shock response associated with hepatocarcinogenesis in a murine model of hereditary tyrosinemia type I.
Hepatocyte injury in tyrosinemia type 1 is induced by fumarylacetoacetate and is inhibited by caspase inhibitors.
Hepatotoxicity and Toxicology of In Vivo Lentiviral Vector Administration in Healthy and Liver-Injury Mouse Models.
Hereditary Tyrosinemia Compounded With Hyperinsulinemic Hypoglycemia: Challenging Diagnosis of a Rare Case.
Hereditary Tyrosinemia Type 1 in Turkey.
Hereditary tyrosinemia type 1 metabolites impair DNA excision repair pathways.
Hereditary tyrosinemia type 1: novel missense, nonsense and splice consensus mutations in the human fumarylacetoacetate hydrolase gene; variability of the genotype-phenotype relationship.
Hereditary tyrosinemia type I-associated mutations in fumarylacetoacetate hydrolase reduce the enzyme stability and increase its aggregation rate.
Hereditary tyrosinemia type I. Self-induced correction of the fumarylacetoacetase defect.
Hereditary tyrosinemia type I: lack of correlation between clinical findings and amount of immunoreactive fumarylacetoacetase protein.
Hereditary tyrosinemia type I: strong association with haplotype 6 in French Canadians permits simple carrier detection and prenatal diagnosis.
Hereditary tyrosinemias (type I): a new vista on tyrosine toxicity and cancer.
High volume naked DNA tail-vein injection restores liver function in Fah-knock out mice.
Hypersuccinylacetonaemia and normal liver function in maleylacetoacetate isomerase deficiency.
Identification of a frequent pseudodeficiency mutation in the fumarylacetoacetase gene, with implications for diagnosis of tyrosinemia type I.
Identification of a stop mutation in five Finnish patients suffering from hereditary tyrosinemia type I.
Identification of mutations causing hereditary tyrosinemia type I in patients of Middle Eastern origin.
Imaging features of type 1 hereditary tyrosinemia: a review of 30 patients.
Impaired DNA repair and genomic stability in hereditary tyrosinemia type 1.
In Vivo Correction of Murine Hereditary Tyrosinemia Type I by ?C31 Integrase-Mediated Gene Delivery.
In vivo correction of murine hereditary tyrosinemia type I by phiC31 integrase-mediated gene delivery.
In vivo selection of hepatocytes transduced with adeno-associated viral vectors.
In vivo suppressor mutations correct a murine model of hereditary tyrosinemia type I.
Infants with Tyrosinemia Type 1: Should phenylalanine be supplemented?
Involvement of endoplasmic reticulum stress in hereditary tyrosinemia type I.
LC-MS/MS method for simultaneous determination on a dried blood spot of multiple analytes relevant for treatment monitoring in patients with tyrosinemia type I.
Liver repopulation and correction of metabolic liver disease by transplanted adult mouse pancreatic cells.
Localization of cells in the rat brain expressing fumarylacetoacetate hydrolase, the deficient enzyme in hereditary tyrosinemia type 1.
Long-term outcome of living donor liver transplantation in a Thai boy with hereditary tyrosinemia type I: a case report.
MicroRNA profiling in lymphocytes and serum of tyrosinemia type-I patients.
Molecular Aspects of the FAH Mutations Involved in HT1 Disease.
Murine fumarylacetoacetate hydrolase (Fah) gene is disrupted by a neonatally lethal albino deletion that defines the hepatocyte-specific developmental regulation 1 (hsdr-1) locus.
Mutation spectrum of fumarylacetoacetase gene and clinical aspects of tyrosinemia type I disease.
Mutational spectrum of Mexican patients with tyrosinemia type 1: In silico modeling and predicted pathogenic effect of a novel missense FAH variant.
Mutations in the fumarylacetoacetate hydrolase gene causing hereditary tyrosinemia type I: overview.
Mutations of the fumarylacetoacetate hydrolase gene in four patients with tyrosinemia, type I.
Neonatal hyperinsulinism in transient and classical forms of tyrosinemia.
Neurocognitive outcome in tyrosinemia type 1 patients compared to healthy controls.
Newborn Screening for Hereditary Tyrosinemia Type I in Québec: Update.
No evidence of maternal cell colonization in reverted liver nodules of tyrosinemia type I patients.
Novel splice, missense, and nonsense mutations in the fumarylacetoacetase gene causing tyrosinemia type 1.
Nucleotide sequence of a cDNA encoding murine fumarylacetoacetate hydrolase.
On the enzymic defects in hereditary tyrosinemia.
Oxidative Stress, Glutathione Metabolism, and Liver Regeneration Pathways Are Activated in Hereditary Tyrosinemia Type 1 Mice upon Short-Term Nitisinone Discontinuation.
Phenotype, genotype, and outcome of 25 Palestinian patients with hereditary tyrosinemia type 1.
Point mutation instability (PIN) mutator phenotype as model for true back mutations seen in hereditary tyrosinemia type 1 - a hypothesis.
Point mutations in the murine fumarylacetoacetate hydrolase gene: Animal models for the human genetic disorder hereditary tyrosinemia type 1.
Prenatal diagnosis of hereditary tyrosinemia by determination of fumarylacetoacetase in cultured amniotic fluid cells.
Presence of three mutations in the fumarylacetoacetate hydrolase gene in a patient with atypical symptoms of hereditary tyrosinemia type I.
Purification of mRNA coding for the enzyme deficient in hereditary tyrosinemia, fumarylacetoacetate hydrolase.
Renal proximal tubular cells acquire resistance to cell death stimuli in mice with hereditary tyrosinemia type 1.
Rescue from neonatal death in the murine model of hereditary tyrosinemia by glutathione monoethylester and vitamin C treatment.
Revisiting hereditary tyrosinemia Type 1-spectrum of radiological findings.
Self-induced correction of the genetic defect in tyrosinemia type I.
Silent Tyrosinemia Type I Without Elevated Tyrosine or Succinylacetone Associated with Liver Cirrhosis and Hepatocellular Carcinoma.
Simple and fast quantification of nitisone (NTBC) using liquid chromatography-tandem mass spectrometry method in plasma of tyrosinemia type 1 patients.
Six novel mutations in the fumarylacetoacetate hydrolase gene of patients with hereditary tyrosinemia type I.
Spectrum of mutations in the fumarylacetoacetate hydrolase gene of tyrosinemia type 1 patients in northwestern Europe and Mediterranean countries.
Splicing mutations, mainly IVS6-1(G>T), account for 70% of fumarylacetoacetate hydrolase (FAH) gene alterations, including 7 novel mutations, in a survey of 29 tyrosinemia type I patients.
Structural and functional analysis of missense mutations in fumarylacetoacetate hydrolase, the gene deficient in hereditary tyrosinemia type 1.
Structural organization and analysis of the human fumarylacetoacetate hydrolase gene in tyrosinemia type I.
Succinylacetone as primary marker to detect tyrosinemia type I in newborns and its measurement by newborn screening programs.
Succinylacetone inhibits delta-aminolevulinate dehydratase and potentiates the drug and steroid induction of delta-aminolevulinate synthase in liver.
Tandem mass spectrometric determination of succinylacetone in dried blood spots enables presymptomatic detection in a case of hepatorenal tyrosinaemia.
Targeting the Apoa1 locus for liver-directed gene therapy.
The Caenorhabditis elegans K10C2.4 Gene Encodes a Member of the Fumarylacetoacetate Hydrolase Family: A CAENORHABDITIS ELEGANS MODEL OF TYPE I TYROSINEMIA.
The effects of early treatment of hereditary tyrosinemia type I in infancy by orthotopic liver transplantation.
The human fumarylacetoacetase gene: characterisation of restriction fragment length polymorphisms and identification of haplotypes in tyrosinemia type 1 and pseudodeficiency.
The kidney in children with tyrosinemia: sonographic, CT and biochemical findings.
The mutagenicity of the tyrosine metabolite, fumarylacetoacetate, is enhanced by glutathione depletion.
The nephropathy of type I tyrosinemia after liver transplantation.
The pathophysiology and treatment of hereditary tyrosinemia type 1.
The pre- and post-natal diagnosis of tyrosinemia type I and the detection of the carrier state by assay of fumarylacetoacetase.
The Unique Spectrum of Mutations in Patients with Hereditary Tyrosinemia Type 1 in Different Regions of the Russian Federation.
Therapeutic genome editing by combined viral and non-viral delivery of CRISPR system components in vivo.
Tissue-specific FAH deficiency alters sleep-wake patterns and results in chronic tyrosinemia in mice.
TRAP-seq identifies cystine/glutamate antiporter as a driver of recovery from liver injury.
Two missense mutations causing tyrosinemia type 1 with presence and absence of immunoreactive fumarylacetoacetase.
Type 1 hereditary tyrosinemia. Evidence for molecular heterogeneity and identification of a causal mutation in a French Canadian patient.
Type I tyrosinemia: lack of immunologically detectable fumarylacetoacetase enzyme protein in tissues and cell extracts.
Tyrosinaemia--treatment and outcome.
Tyrosinemia type 1 and irreversible neurologic crisis after one month discontinuation of nitisone.
Tyrosinemia type 1 should be suspected in infants with severe coagulopathy even in the absence of other signs of liver failure.
Tyrosinemia type 1--complex splicing defects and a missense mutation in the fumarylacetoacetase gene.
Tyrosinemia type 1: a rare and forgotten cause of reversible hypertrophic cardiomyopathy in infancy.
Tyrosinemia type I and not treatment with NTBC causes slower learning and altered behavior in mice.
Tyrosinemia type I: a clinico-laboratory case report.
Tyrosinemia Typel: A case report.
Tyrosinemia: the Quebec experience.
Urinary excretion of deuterated metabolites in patients with tyrosinemia type I after oral loading with deuterated L-tyrosine.
Urinary excretion of succinylacetone and delta-aminolevulinic acid in patients with hereditary tyrosinemia.
Variable gene expression within human tyrosinemia type 1 liver may reflect region-specific dysplasia.
[Clinical, biochemical and molecular characteristics in 11 Czech children with tyrosinemia type I].
[delta-Aminolevulinate dehydratase deficiency]
[Evolution of a case of tyrosinemia type I treated with NTBC]
[Juvenile form of tyrosinemia type I]
Urinary Bladder Neoplasms
Dose-dependent reduction of N-2-fluorenylacetamide-induced liver cancer and enhancement of bladder cancer in rats by butylated hydroxytoluene.
Vasculitis
The role of colour doppler ultrasonography of facial and occipital arteries in patients with giant cell arteritis: A prospective study.
Venous Thrombosis
Treatment with covered stent of giant femoral artery aneurysm causing deep vein thrombosis.
Vertigo
Benign paroxysmal positional vertigo in an airline pilot.