Disease on EC 2.6.1.13 - ornithine aminotransferase
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arginase deficiency
Biomarkers identified in inborn errors for lysine, arginine, and ornithine.
aromatic-l-amino-acid decarboxylase deficiency
Targeted cerebrospinal fluid analysis for inborn errors of metabolism on an LC-MS/MS analysis platform.
Blindness
A 15-bp deletion in exon 5 of the ornithine aminotransferase (OAT) locus associated with gyrate atrophy.
Blindness
Deficit of human ornithine aminotransferase in gyrate atrophy: Molecular, cellular, and clinical aspects.
Blindness
First report of c.425-1G>A mutation in ornithine aminotransferase gene causing gyrate atrophy of the choroid and retina with hyperornithinemia.
Blindness
Gyrate atrophy of the choroid and retina with hyperornithinemia: characterization of mutant liver L-ornithine:2-oxoacid aminotransferase kinetics.
Blindness
Oligomeric State and Thermal Stability of Apo- and Holo- Human Ornithine ?-Aminotransferase.
Blindness
Point mutation affecting processing of the ornithine aminotransferase precursor protein in gyrate atrophy.
Blindness
Retrovirus-mediated gene transfer of ornithine-delta-aminotransferase into keratinocytes from gyrate atrophy patients.
Brain Diseases
Effects of inhibition of ornithine aminotransferase on thioacetamide-induced hepatogenic encephalopathy.
Carcinogenesis
[Expression and Bioinformatic Analysis of Ornithine Aminotransferase ?in Non-small Cell Lung Cancer].
Carcinoma
Identification of a yeast artificial chromosome that spans the human papillary renal cell carcinoma-associated t(X;1) breakpoint in Xp11.2.
Carcinoma
Proteome of Metastatic Canine Mammary Carcinomas: Similarities to and Differences from Human Breast Cancer ().
Carcinoma, Hepatocellular
A Remarkable Difference That One Fluorine Atom Confers on the Mechanisms of Inactivation of Human Ornithine Aminotransferase by Two Cyclohexene Analogues of ?-Aminobutyric Acid.
Carcinoma, Hepatocellular
Establishment of a clonal strain of hepatoma cells derived from Morris hepatoma 8999.
Carcinoma, Hepatocellular
Expression mechanism of abnormality of ornithine aminotransferase level in Morris hepatomas.
Carcinoma, Hepatocellular
Imbalance in ornithine metabolism in hepatomas of different growth rates as expressed in behavior of L-ornithine:2-oxoacid aminotransferase (ornithine transaminase, EC 2.6.1.13).
Carcinoma, Hepatocellular
Mechanism of Inactivation of Ornithine Aminotransferase by (1S,3S)-3-Amino-4-(hexafluoropropan-2-ylidenyl)cyclopentane-1-carboxylic Acid.
Carcinoma, Hepatocellular
Remarkable and Unexpected Mechanism for (S)-3-Amino-4-(difluoromethylenyl)cyclohex-1-ene-1-carboxylic Acid as a Selective Inactivator of Human Ornithine Aminotransferase.
Carcinoma, Hepatocellular
Studies on the turnover rates of ornithine aminotransferase in Morris hepatoma 44 and host liver.
Carcinoma, Hepatocellular
Suppression of Hepatocellular Carcinoma by Inhibition of Overexpressed Ornithine Aminotransferase.
Carcinoma, Non-Small-Cell Lung
Ornithine aminotransferase promoted the proliferation and metastasis of non-small cell lung cancer via upregulation of miR-21.
Carcinoma, Renal Cell
Identification of a yeast artificial chromosome that spans the human papillary renal cell carcinoma-associated t(X;1) breakpoint in Xp11.2.
Colonic Neoplasms
Analysis of differentially expressed genes in human hepatocellular carcinoma using suppression subtractive hybridization.
Crigler-Najjar Syndrome
Successful living donor liver transplantation plus domino-auxiliary partial orthotopic liver transplantation for pediatric patients with metabolic disorders.
Dehydration
Effect of drought on polyamine metabolism, yield, protein content and in vitro protein digestibility in tepary (Phaseolus acutifolius) and common (Phaseolus vulgaris) bean seeds
Dehydration
Water deficit-induced oxidative stress affects artemisinin content and expression of proline metabolic genes in Artemisia annua L.
Endotoxemia
Adaptative response of nitrogen metabolism in early endotoxemia: role of ornithine aminotransferase.
Eye Diseases
A single-base change at a splice acceptor site in the ornithine aminotransferase gene causes abnormal RNA splicing in gyrate atrophy.
Eye Diseases
Molecular basis of ornithine aminotransferase deficiency in B-6-responsive and -nonresponsive forms of gyrate atrophy.
Eye Diseases
Splicing defect at the ornithine aminotransferase (OAT) locus in gyrate atrophy.
guanidinoacetate n-methyltransferase deficiency
Targeted cerebrospinal fluid analysis for inborn errors of metabolism on an LC-MS/MS analysis platform.
Gyrate Atrophy
A 15-bp deletion in exon 5 of the ornithine aminotransferase (OAT) locus associated with gyrate atrophy.
Gyrate Atrophy
A mouse model of gyrate atrophy of the choroid and retina. Early retinal pigment epithelium damage and progressive retinal degeneration.
Gyrate Atrophy
A single amino acid substitution within the mature sequence of ornithine aminotransferase obstructs mitochondrial entry of the precursor.
Gyrate Atrophy
A single-base change at a splice acceptor site in the ornithine aminotransferase gene causes abnormal RNA splicing in gyrate atrophy.
Gyrate Atrophy
An initiator codon mutation in ornithine-delta-aminotransferase causing gyrate atrophy of the choroid and retina.
Gyrate Atrophy
At least two mutant alleles of ornithine delta-aminotransferase cause gyrate atrophy of the choroid and retina in Finns.
Gyrate Atrophy
Bevacizumab for the treatment of intraretinal cystic spaces in a patient with gyrate atrophy of the choroid and retina.
Gyrate Atrophy
Carbonic anhydrase inhibitor with topical NSAID therapy to manage cystoid macular edema in a case of gyrate atrophy.
Gyrate Atrophy
Central nervous system involvement in gyrate atrophy of the choroid and retina with hyperornithinaemia.
Gyrate Atrophy
Chromosomal localization of human ornithine aminotransferase gene sequences to 10q26 and Xp11.2.
Gyrate Atrophy
Comparison of ornithine metabolism in hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome, lysinuric protein intolerance and gyrate atrophy fibroblasts.
Gyrate Atrophy
Correction of ornithine accumulation prevents retinal degeneration in a mouse model of gyrate atrophy of the choroid and retina.
Gyrate Atrophy
Correction of ornithine-delta-aminotransferase deficiency in a Chinese hamster ovary cell line mediated by retrovirus gene transfer.
Gyrate Atrophy
Cystoid macular edema secondary to gyrate atrophy in a child treated with sub-tenon injection of triamcinolone acetonide.
Gyrate Atrophy
Defective ornithine metabolism in cultured skin fibroblasts from patients with the syndrome of hyperornithinemia, hyperammonemia and homocitrullinuria.
Gyrate Atrophy
Deficit of human ornithine aminotransferase in gyrate atrophy: Molecular, cellular, and clinical aspects.
Gyrate Atrophy
Detection of point mutations associated with genetic diseases by an exon scanning technique.
Gyrate Atrophy
Expression and processing of human ornithine-delta-aminotransferase in Saccharomyces cerevisiae.
Gyrate Atrophy
Expression of human ornithine aminotransferase (OAT) in OAT-deficient Chinese hamster ovary cells and fibroblasts of gyrate atrophy patient.
Gyrate Atrophy
First report of c.425-1G>A mutation in ornithine aminotransferase gene causing gyrate atrophy of the choroid and retina with hyperornithinemia.
Gyrate Atrophy
Functional analysis of missense mutations of OAT, causing gyrate atrophy of choroid and retina.
Gyrate Atrophy
Genotype-phenotype correlation of a pyridoxine-responsive form of gyrate atrophy.
Gyrate Atrophy
Gyrate atrophy of the choroid and retina with hyperornithinemia. Deficient formation of guanidinoacetic acid from arginine.
Gyrate Atrophy
Gyrate atrophy of the choroid and retina with hyperornithinemia: characterization of mutant liver L-ornithine:2-oxoacid aminotransferase kinetics.
Gyrate Atrophy
Gyrate atrophy of the choroid and retina. A five-year follow-up of creatine supplementation.
Gyrate Atrophy
Gyrate atrophy of the choroid and retina. Biochemical considerations and experience with an arginine-restricted diet.
Gyrate Atrophy
Gyrate atrophy of the choroid and retina: assignment of the ornithine aminotransferase structural gene to human chromosome 10 and mouse chromosome 7.
Gyrate Atrophy
Gyrate atrophy of the choroid and retina: characterization of mutant ornithine aminotransferase and mechanism of response to vitamin B6.
Gyrate Atrophy
Gyrate atrophy of the choroid and retina: decreased ornithine aminotransferase concentration in cultured skin fibroblasts from patients.
Gyrate Atrophy
Gyrate atrophy of the choroid and retina: deficiency of ornithine aminotransferase in transformed lymphocytes.
Gyrate Atrophy
Gyrate atrophy of the choroid and retina: lymphocyte ornithine-delta-aminotransferase activity in different mutations and carriers.
Gyrate Atrophy
Gyrate atrophy of the retina and choroid is an inborn error of metabolism of ornithine aminotransferase.
Gyrate Atrophy
Gyrate atrophy of the retina and choroid. Two methods for prenatal diagnosis.
Gyrate Atrophy
Heterogeneity and uniqueness of ornithine aminotransferase mutations found in Japanese gyrate atrophy patients.
Gyrate Atrophy
Heterogeneity in ornithine cytotoxicity of bovine retinal pigment epithelial cells in primary culture.
Gyrate Atrophy
Immunocytochemical localization of ornithine aminotransferase in human ocular tissues.
Gyrate Atrophy
Immunocytochemical localization of ornithine aminotransferase in rat ocular tissues.
Gyrate Atrophy
Immunohistochemical localization of ornithine aminotransferase in normal rat tissues by Fab'-horseradish peroxidase conjugates.
Gyrate Atrophy
Induction of arginase II mRNA by nitric oxide using in vitro model of gyrate atrophy of choroid and retina.
Gyrate Atrophy
Inheritance of ornithine aminotransferase gene, mRNA, and enzyme defect in a family with gyrate atrophy of the choroid and retina.
Gyrate Atrophy
Inhibition of arginine-glycine amidinotransferase by ornithine. A possible mechanism for the muscular and chorioretinal atrophies in gyrate atrophy of the choroid and retina with hyperornithinemia.
Gyrate Atrophy
Investigation of gyrate atrophy using a cDNA clone for human ornithine aminotransferase.
Gyrate Atrophy
Mapping of ornithine aminotransferase gene sequences to mouse chromosomes 7, X, and 3.
Gyrate Atrophy
Metabolic follow-up of a Croatian patient with gyrate atrophy and a new mutation in the OAT gene: a case report.
Gyrate Atrophy
Mice lacking ornithine-delta-aminotransferase have paradoxical neonatal hypoornithinaemia and retinal degeneration.
Gyrate Atrophy
Molecular and Cellular Studies Reveal Folding Defects of Human Ornithine Aminotransferase Variants Associated With Gyrate Atrophy of the Choroid and Retina.
Gyrate Atrophy
Molecular basis of ornithine aminotransferase deficiency in B-6-responsive and -nonresponsive forms of gyrate atrophy.
Gyrate Atrophy
Molecular pathology of gyrate atrophy of the choroid and retina due to ornithine aminotransferase deficiency.
Gyrate Atrophy
Nonsense-codon mutations of the ornithine aminotransferase gene with decreased levels of mutant mRNA in gyrate atrophy.
Gyrate Atrophy
Oligomeric State and Thermal Stability of Apo- and Holo- Human Ornithine ?-Aminotransferase.
Gyrate Atrophy
Ophthalmologic heterogeneity in subjects with gyrate atrophy of choroid and retina harboring the L402P mutation of ornithine aminotransferase.
Gyrate Atrophy
Ornithine Aminotransferase Deficiency in Differential Diagnosis of Neonatal Hyperammonemia: A Case with a Novel OAT Gene Mutation.
Gyrate Atrophy
Ornithine aminotransferase deficiency: diagnostic difficulties in neonatal presentation.
Gyrate Atrophy
Ornithine aminotransferase distribution in ocular tissues and retinas of cat and mouse.
Gyrate Atrophy
Ornithine delta-aminotransferase mutations in gyrate atrophy. Allelic heterogeneity and functional consequences.
Gyrate Atrophy
Ornithine transport via cationic amino acid transporter-1 is involved in ornithine cytotoxicity in retinal pigment epithelial cells.
Gyrate Atrophy
Ornithine-delta-aminotransferase expression and ornithine metabolism in cultured epidermal keratinocytes: toward metabolic sink therapy for gyrate atrophy.
Gyrate Atrophy
Pathology of iridectomy specimens in gyrate atrophy of the retina and choroid.
Gyrate Atrophy
Point mutation affecting processing of the ornithine aminotransferase precursor protein in gyrate atrophy.
Gyrate Atrophy
Prevention of ornithine cytotoxicity by nonpolar side chain amino acids in retinal pigment epithelial cells.
Gyrate Atrophy
Prevention of ornithine cytotoxicity by proline in human retinal pigment epithelial cells.
Gyrate Atrophy
Pyridoxine-responsive gyrate atrophy of the choroid and retina: clinical and biochemical correlates of the mutation A226V.
Gyrate Atrophy
Rapid and efficient molecular analysis of gyrate atrophy using denaturing gradient gel electrophoresis.
Gyrate Atrophy
Regression of macular edema with topical brinzolamide and nepafenac alone and identification of a novel gyrate atrophy mutation.
Gyrate Atrophy
Regulation of proline biosynthesis: the inhibition of pyrroline-5-carboxylate synthase activity by ornithine.
Gyrate Atrophy
Retrovirus-mediated gene transfer of ornithine-delta-aminotransferase into keratinocytes from gyrate atrophy patients.
Gyrate Atrophy
Secondary creatine deficiency in ornithine delta-aminotransferase deficiency.
Gyrate Atrophy
Splicing defect at the ornithine aminotransferase (OAT) locus in gyrate atrophy.
Gyrate Atrophy
Successful living donor liver transplantation plus domino-auxiliary partial orthotopic liver transplantation for pediatric patients with metabolic disorders.
Gyrate Atrophy
Targeted cerebrospinal fluid analysis for inborn errors of metabolism on an LC-MS/MS analysis platform.
Gyrate Atrophy
The ornithine aminotransferase (OAT) locus: analysis of RFLPs in gyrate atrophy.
Gyrate Atrophy
The ornithine aminotransferase gene in gyrate atrophy of the retina: analysis of expression and gross structure of this gene in cultured fibroblasts.
Gyrate Atrophy
Three novel mutations of the ornithine aminotransferase (OAT) gene in gyrate atrophy.
Gyrate Atrophy
Vitamin B6-responsive ornithine aminotransferase deficiency with a novel mutation G237D.
Gyrate Atrophy
[Hyperornithinemia and gyrate atrophy: ornithine aminotransferase gene error causing a Finnish disease]
Gyrate Atrophy
[Molecular cloning of the genes in genetic chorioretinal diseases--positional cloning and the candidate gene approach]
Hepatomegaly
Liver-targeted disruption of Apc in mice activates beta-catenin signaling and leads to hepatocellular carcinomas.
Hyperargininemia
Biomarkers identified in inborn errors for lysine, arginine, and ornithine.
Hyperthyroidism
Role of thyroxine in the postnatal development of rat hepatic tryptophan oxygenase and ornithine aminotransferase.
Infections
Helicobacter pylori in a Korean Isolate Expressed Proteins Differentially in Human Gastric Epithelial Cells.
Infections
Interactions of ibuprofen with influenza infection and hyperammonemia in an animal model of Reye's syndrome.
Infections
Metabolic adjustments during compatible interaction between barley genotypes and stripe rust pathogen.
Influenza, Human
Interactions of ibuprofen with influenza infection and hyperammonemia in an animal model of Reye's syndrome.
Liver Neoplasms
Mechanism of Inactivation of Ornithine Aminotransferase by (1S,3S)-3-Amino-4-(hexafluoropropan-2-ylidenyl)cyclopentane-1-carboxylic Acid.
Liver Neoplasms, Experimental
Studies on the turnover rates of ornithine aminotransferase in Morris hepatoma 44 and host liver.
Lung Neoplasms
Ornithine aminotransferase promoted the proliferation and metastasis of non-small cell lung cancer via upregulation of miR-21.
Lung Neoplasms
Quantitative proteomic analysis of mitochondrial proteins differentially expressed between small cell lung cancer cells and normal human bronchial epithelial cells.
Lymphoma
Tissue enzyme changes in parabiotic rats with subcutaneous lymphoma or fibrosarcoma.
Metabolic Diseases
Cystoid macular edema secondary to gyrate atrophy in a child treated with sub-tenon injection of triamcinolone acetonide.
methylenetetrahydrofolate dehydrogenase (nad+) deficiency
Targeted cerebrospinal fluid analysis for inborn errors of metabolism on an LC-MS/MS analysis platform.
Neoplasm Metastasis
Ornithine aminotransferase promoted the proliferation and metastasis of non-small cell lung cancer via upregulation of miR-21.
Neoplasm Metastasis
Quantitative proteomic analysis of mitochondrial proteins differentially expressed between small cell lung cancer cells and normal human bronchial epithelial cells.
Neoplasms
Breast Cancer-Derived Microvesicles Are the Source of Functional Metabolic Enzymes as Potential Targets for Cancer Therapy.
Neoplasms
Identification of differentially expressed proteins in spontaneous thymic lymphomas from knockout mice with deletion of p53.
Neoplasms
Mapping Metabolic Events in the Cancer Cell Cycle Reveals Arginine Catabolism in the Committed SG2M Phase.
Neoplasms
Ornithine aminotransferase promoted the proliferation and metastasis of non-small cell lung cancer via upregulation of miR-21.
Neoplasms
Quantitative proteomic analysis of mitochondrial proteins differentially expressed between small cell lung cancer cells and normal human bronchial epithelial cells.
Neoplasms
The effect of lymphoma and other neoplasms on hepatic and plasma enzymes of the host rat.
Neoplasms
[Expression and Bioinformatic Analysis of Ornithine Aminotransferase ?in Non-small Cell Lung Cancer].
Neurodegenerative Diseases
Proteome response to ochratoxin A-induced apoptotic cell death in mouse hippocampal HT22 cells.
Non-alcoholic Fatty Liver Disease
New Potential Biomarker Proteins for Alcoholic Liver Disease Identified by a Comparative Proteomics Approach.
ornithine aminotransferase deficiency
A new sensitive and convenient assay of ornithine aminotransferase.
ornithine aminotransferase deficiency
Biomarkers identified in inborn errors for lysine, arginine, and ornithine.
ornithine aminotransferase deficiency
Correction of ornithine-delta-aminotransferase deficiency in a Chinese hamster ovary cell line mediated by retrovirus gene transfer.
ornithine aminotransferase deficiency
Dietary compliance in ornithine aminotransferase deficiency.
ornithine aminotransferase deficiency
Gyrate atrophy of the choroid and retina in a 5-year-old girl.
ornithine aminotransferase deficiency
Gyrate atrophy of the choroid and retina. Biochemical considerations and experience with an arginine-restricted diet.
ornithine aminotransferase deficiency
Hyperornithinaemia and gyrate atrophy of the retina: improvement of vision during treatment with a low-arginine diet.
ornithine aminotransferase deficiency
Molecular basis of ornithine aminotransferase deficiency in B-6-responsive and -nonresponsive forms of gyrate atrophy.
ornithine aminotransferase deficiency
Molecular pathology of gyrate atrophy of the choroid and retina due to ornithine aminotransferase deficiency.
ornithine aminotransferase deficiency
Ornithine Aminotransferase Deficiency in Differential Diagnosis of Neonatal Hyperammonemia: A Case with a Novel OAT Gene Mutation.
ornithine aminotransferase deficiency
Ornithine aminotransferase deficiency: diagnostic difficulties in neonatal presentation.
ornithine aminotransferase deficiency
Pathology of iridectomy specimens in gyrate atrophy of the retina and choroid.
ornithine aminotransferase deficiency
Secondary creatine deficiency in ornithine delta-aminotransferase deficiency.
ornithine aminotransferase deficiency
Successful living donor liver transplantation plus domino-auxiliary partial orthotopic liver transplantation for pediatric patients with metabolic disorders.
ornithine aminotransferase deficiency
Sustained oral lysine supplementation in ornithine delta-aminotransferase deficiency.
ornithine aminotransferase deficiency
Targeted cerebrospinal fluid analysis for inborn errors of metabolism on an LC-MS/MS analysis platform.
ornithine aminotransferase deficiency
Vitamin B6-responsive ornithine aminotransferase deficiency with a novel mutation G237D.
ornithine aminotransferase deficiency
[Fuchs' gyrate atrophy and ornithine aminotransferase deficiency]
Renal Insufficiency
Messenger RNA for enzymes of ornithine and polyamine metabolism are selectively underexpressed in kidney of 5/6 nephrectomized rats.
Retinal Degeneration
Gyrate atrophy of the retina and choroid is an inborn error of metabolism of ornithine aminotransferase.
Retinal Degeneration
Mice lacking ornithine-delta-aminotransferase have paradoxical neonatal hypoornithinaemia and retinal degeneration.
Retinal Degeneration
Pathology of iridectomy specimens in gyrate atrophy of the retina and choroid.
Sarcoma, Synovial
A synovial sarcoma with a complex t(X;18;5;4) and a break in the ornithine aminotransferase (OAT)L1 cluster on Xp11.2.
Sarcoma, Synovial
Identification of a yeast artificial chromosome that spans the human papillary renal cell carcinoma-associated t(X;1) breakpoint in Xp11.2.
Seizures
Enhanced endogenous ornithine concentrations protect against tonic seizures and coma in acute ammonia intoxication.
Seizures
Functional significance of the activities of glutaminase and ornithine-?-aminotransferase in rat brain.
Squamous Cell Carcinoma of Head and Neck
Modulation of L-Arginine-Arginase Metabolic Pathway Enzymes: Immunocytochemistry and mRNA Expression in Peripheral Blood and Tissue Levels in Head and Neck Squamous Cell Carcinomas in North East India.
Starvation
Effects of cortisol or starvation on the activities of four enzymes in small intestine and liver of the rat during development.
Tuberculosis
Mycobacterium tuberculosis Is a Natural Ornithine Aminotransferase (rocD) Mutant and Depends on Rv2323c for Growth on Arginine.
Vitamin B 6 Deficiency
Effect of pyridoxine deficiency on ornithine aminotransferase in rat kidney and liver.
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