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Adenocarcinoma
Reduced expression of argininosuccinate synthetase 1 has a negative prognostic impact in patients with pancreatic ductal adenocarcinoma.
Adenoma, Liver Cell
Argininosuccinate synthase 1 (ASS1): A marker of unclassified hepatocellular adenoma and high bleeding risk.
Alzheimer Disease
Neuronal and glial coexpression of argininosuccinate synthetase and inducible nitric oxide synthase in Alzheimer disease.
amino-acid n-acetyltransferase deficiency
Genetic approach to prenatal diagnosis in urea cycle defects.
amino-acid n-acetyltransferase deficiency
Improving long term outcomes in urea cycle disorders-report from the Urea Cycle Disorders Consortium.
Amyloid Neuropathies, Familial
[Recent advances of the treatment in metabolic disorders]
arginase deficiency
Biochemical markers and neuropsychological functioning in distal urea cycle disorders.
arginase deficiency
Genetic approach to prenatal diagnosis in urea cycle defects.
arginase deficiency
Improving long term outcomes in urea cycle disorders-report from the Urea Cycle Disorders Consortium.
arginase deficiency
Long-term outcome of urea cycle disorders: Report from a nationwide study in Japan.
arginase deficiency
Physical, cognitive, and social status of patients with urea cycle disorders in Japan.
arginase deficiency
Role of liver transplantation in urea cycle disorders: Report from a nationwide study in Japan.
argininosuccinate lyase deficiency
Ammonia Control in Children Ages 2 Months through 5 Years with Urea Cycle Disorders: Comparison of Sodium Phenylbutyrate and Glycerol Phenylbutyrate.
argininosuccinate lyase deficiency
Biochemical markers and neuropsychological functioning in distal urea cycle disorders.
argininosuccinate lyase deficiency
Clinical and genetic diversity of congenital hyperammonemia.
argininosuccinate lyase deficiency
Complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts.
argininosuccinate lyase deficiency
Decreased plasma l-arginine levels in organic acidurias (MMA and PA) and decreased plasma branched-chain amino acid levels in urea cycle disorders as a potential cause of growth retardation: Options for treatment.
argininosuccinate lyase deficiency
Genetic approach to prenatal diagnosis in urea cycle defects.
argininosuccinate lyase deficiency
Impact of Diagnosis and Therapy on Cognitive Function in Urea Cycle Disorders.
argininosuccinate lyase deficiency
Improving long term outcomes in urea cycle disorders-report from the Urea Cycle Disorders Consortium.
argininosuccinate lyase deficiency
Long-term outcome of urea cycle disorders: Report from a nationwide study in Japan.
argininosuccinate lyase deficiency
Physical, cognitive, and social status of patients with urea cycle disorders in Japan.
argininosuccinate lyase deficiency
Prospective treatment of urea cycle disorders.
argininosuccinate lyase deficiency
Studies on complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts.
argininosuccinate lyase deficiency
Urea cycle disorders in Argentine patients: clinical presentation, biochemical and genetic findings.
argininosuccinate synthase deficiency
A GC/MS-based metabolomic approach for diagnosing citrin deficiency.
argininosuccinate synthase deficiency
A novel inborn error of metabolism detected by elevated methionine and/or galactose in newborn screening: neonatal intrahepatic cholestasis caused by citrin deficiency.
argininosuccinate synthase deficiency
A patient with type II citrullinemia who developed refractory complex seizure.
argininosuccinate synthase deficiency
Ammonia Control in Children Ages 2 Months through 5 Years with Urea Cycle Disorders: Comparison of Sodium Phenylbutyrate and Glycerol Phenylbutyrate.
argininosuccinate synthase deficiency
An adult-onset case of argininosuccinate synthetase deficiency presenting with atypical citrullinemia.
argininosuccinate synthase deficiency
Argininosuccinate synthetase deficiency and reye syndrome-like presentation.
argininosuccinate synthase deficiency
Argininosuccinate synthetase deficiency: mutation analysis in 3 Thai patients.
argininosuccinate synthase deficiency
Argininosuccinic acid synthetase deficiency in a hamster cell line and its complementation of argininosuccinic aciduria human fibroblasts.
argininosuccinate synthase deficiency
Biochemical markers and neuropsychological functioning in distal urea cycle disorders.
argininosuccinate synthase deficiency
Citrullinemia type I and hypertrophic pyloric stenosis in a 1-month old male infant.
argininosuccinate synthase deficiency
Citrullinemia with an atypical presentation: persistent hiccups. Case report.
argininosuccinate synthase deficiency
Citrullinemia, argininosuccinate synthetase deficiency. Repository identification No. GM-1044.
argininosuccinate synthase deficiency
Clearance of amino acids by hemodialysis in argininosuccinate synthetase deficiency.
argininosuccinate synthase deficiency
Clinical and biochemical characteristics of patients with urea cycle disorders in a developing country.
argininosuccinate synthase deficiency
Clinical and genetic diversity of congenital hyperammonemia.
argininosuccinate synthase deficiency
Decreased plasma l-arginine levels in organic acidurias (MMA and PA) and decreased plasma branched-chain amino acid levels in urea cycle disorders as a potential cause of growth retardation: Options for treatment.
argininosuccinate synthase deficiency
Effects of glucagon on urinary excretion of urea and on plasma ammonia level in argininosuccinate synthetase deficiency.
argininosuccinate synthase deficiency
Extracellular vesicles from human liver stem cells restore argininosuccinate synthase deficiency.
argininosuccinate synthase deficiency
Favorable long-term outcome following severe neonatal hyperammonemic coma in a patient with argininosuccinate synthetase deficiency.
argininosuccinate synthase deficiency
First report of carglumic acid in a patient with citrullinemia type 1 (argininosuccinate synthetase deficiency).
argininosuccinate synthase deficiency
Genetic approach to prenatal diagnosis in urea cycle defects.
argininosuccinate synthase deficiency
Impaired T cell function in argininosuccinate synthetase deficiency.
argininosuccinate synthase deficiency
Improving long term outcomes in urea cycle disorders-report from the Urea Cycle Disorders Consortium.
argininosuccinate synthase deficiency
In vivo urea cycle flux distinguishes and correlates with phenotypic severity in disorders of the urea cycle.
argininosuccinate synthase deficiency
Incidence and distribution of argininosuccinate synthetase deficiency in human cancers: a method for identifying cancers sensitive to arginine deprivation.
argininosuccinate synthase deficiency
Infantile cholestatic jaundice associated with adult-onset type II citrullinemia.
argininosuccinate synthase deficiency
Kinetic mutations in argininosuccinate synthetase deficiency: characterisation and in vitro correction by substrate supplementation.
argininosuccinate synthase deficiency
Letter: The high levels of lysine, homocitrulline, and homoarginine found in argininosuccinate synthetase deficiency.
argininosuccinate synthase deficiency
Letter: The presence of the homoanalogues of substrates of the urea cycle in the presence of argininosuccinate synthetase deficiency.
argininosuccinate synthase deficiency
Liver transplantation may prevent neurodevelopmental deterioration in high-risk patients with urea cycle disorders.
argininosuccinate synthase deficiency
Living-related liver transplantation for type II citrullinemia using a graft from heterozygote donor.
argininosuccinate synthase deficiency
Long-term outcome of urea cycle disorders: Report from a nationwide study in Japan.
argininosuccinate synthase deficiency
Long-term survival of patients with argininosuccinate synthetase deficiency.
argininosuccinate synthase deficiency
Mild citrullinemia in Caucasians is an allelic variant of argininosuccinate synthetase deficiency (citrullinemia type 1).
argininosuccinate synthase deficiency
Molecular analysis of argininosuccinate synthetase deficiency in human fibroblasts.
argininosuccinate synthase deficiency
Molecular definition of bovine argininosuccinate synthetase deficiency.
argininosuccinate synthase deficiency
Neonatal presentation of adult-onset type II citrullinemia.
argininosuccinate synthase deficiency
Neonatal type of argininosuccinate synthetase deficiency. Report of two cases with autopsy findings.
argininosuccinate synthase deficiency
Orthotopic liver transplantation for urea cycle enzyme deficiency.
argininosuccinate synthase deficiency
Physical, cognitive, and social status of patients with urea cycle disorders in Japan.
argininosuccinate synthase deficiency
Plasma concentrations and renal clearance of orotic acid in argininosuccinic acid synthetase deficiency.
argininosuccinate synthase deficiency
Postpartum "psychosis" in mild argininosuccinate synthetase deficiency.
argininosuccinate synthase deficiency
Pregnancy in a healthy woman with untreated citrullinemia.
argininosuccinate synthase deficiency
Prenatal diagnosis of citrullinemia and argininosuccinic aciduria: evidence for a transmission ratio distortion in citrullinemia.
argininosuccinate synthase deficiency
Prenatal diagnosis of citrullinemia type 1: A Chinese family with a novel mutation of the ASS1 gene.
argininosuccinate synthase deficiency
Prospective treatment of urea cycle disorders.
argininosuccinate synthase deficiency
Role of liver transplantation in urea cycle disorders: Report from a nationwide study in Japan.
argininosuccinate synthase deficiency
Studies on complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts.
argininosuccinate synthase deficiency
The Diagnosis and Management of a Female With Mild Citrullinemia and Undetectable Argininosuccinate Synthetase Activity in Fibroblasts.
argininosuccinate synthase deficiency
Transiently reduced activity of carbamyl phosphate synthetase and ornithine transcarbamylase in liver of children with Reye's syndrome.
argininosuccinate synthase deficiency
Urea cycle disorders in Argentine patients: clinical presentation, biochemical and genetic findings.
argininosuccinate synthase deficiency
Urea cycle disorders in Thai infants: a report of 5 cases.
argininosuccinate synthase deficiency
[Argininosuccinate synthetase deficiency]
Argininosuccinic Aciduria
Ammonia Control in Children Ages 2 Months through 5 Years with Urea Cycle Disorders: Comparison of Sodium Phenylbutyrate and Glycerol Phenylbutyrate.
Argininosuccinic Aciduria
Argininosuccinic acid synthetase deficiency in a hamster cell line and its complementation of argininosuccinic aciduria human fibroblasts.
Argininosuccinic Aciduria
Biochemical markers and neuropsychological functioning in distal urea cycle disorders.
Argininosuccinic Aciduria
Clinical and genetic diversity of congenital hyperammonemia.
Argininosuccinic Aciduria
Complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts.
Argininosuccinic Aciduria
Decreased plasma l-arginine levels in organic acidurias (MMA and PA) and decreased plasma branched-chain amino acid levels in urea cycle disorders as a potential cause of growth retardation: Options for treatment.
Argininosuccinic Aciduria
Genetic approach to prenatal diagnosis in urea cycle defects.
Argininosuccinic Aciduria
Impact of Diagnosis and Therapy on Cognitive Function in Urea Cycle Disorders.
Argininosuccinic Aciduria
Improving long term outcomes in urea cycle disorders-report from the Urea Cycle Disorders Consortium.
Argininosuccinic Aciduria
Long-term outcome of urea cycle disorders: Report from a nationwide study in Japan.
Argininosuccinic Aciduria
Physical, cognitive, and social status of patients with urea cycle disorders in Japan.
Argininosuccinic Aciduria
Prospective treatment of urea cycle disorders.
Argininosuccinic Aciduria
Studies on complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts.
Argininosuccinic Aciduria
Urea cycle disorders in Argentine patients: clinical presentation, biochemical and genetic findings.
Arthritis
Induction of inducible nitric oxide synthase, argininosuccinate synthase, and GTP cyclohydrolase I in arthritic joints of human tumor necrosis factor-alpha transgenic mice.
Bacterial Infections
Inhibition of LPS toxicity by hepatic argininosuccinate synthase (ASS): Novel roles for ASS in innate immune responses to bacterial infection.
Brain Diseases
Auxiliary partial orthotopic liver transplantation for adult onset type II citrullinemia.
Brain Diseases
Citrullinemia with an atypical presentation: persistent hiccups. Case report.
Brain Diseases
Hyperammonaemic encephalopathy in an adult patient with citrin deficiency associated with a novel mutation.
Breast Neoplasms
Arginine depriving enzymes: applications as emerging therapeutics in cancer treatment.
carbamoyl-phosphate synthase (ammonia) deficiency
Increased excretion of N-carbamoyl compounds in patients with urea cycle defects.
carbamoyl-phosphate synthase (ammonia) deficiency
Liver transplantation may prevent neurodevelopmental deterioration in high-risk patients with urea cycle disorders.
carbamoyl-phosphate synthase (ammonia) deficiency
Physical, cognitive, and social status of patients with urea cycle disorders in Japan.
carbamoyl-phosphate synthase (ammonia) deficiency
Prospective treatment of urea cycle disorders.
carbamoyl-phosphate synthase (ammonia) deficiency
Role of liver transplantation in urea cycle disorders: Report from a nationwide study in Japan.
Carcinogenesis
Aberrant regulation of argininosuccinate synthetase by TNF-alpha in human epithelial ovarian cancer.
Carcinogenesis
Increased expression of argininosuccinate synthetase protein predicts poor prognosis in human gastric cancer.
Carcinoma
Androgen receptor regulates ASS1P3/miR-34a-5p/ASS1 signaling to promote renal cell carcinoma cell growth.
Carcinoma
Differential expression of argininosuccinate synthetase in serous and non-serous ovarian carcinomas.
Carcinoma
Renal cell carcinoma does not express argininosuccinate synthetase and is highly sensitive to arginine deprivation via arginine deiminase.
Carcinoma
Systems level profiling of arginine starvation reveals MYC and ERK adaptive metabolic reprogramming.
Carcinoma, Hepatocellular
A randomised phase II study of pegylated arginine deiminase (ADI-PEG 20) in Asian advanced hepatocellular carcinoma patients.
Carcinoma, Hepatocellular
Arginine deiminase augments the chemosensitivity of argininosuccinate synthetase-deficient pancreatic cancer cells to gemcitabine via inhibition of NF-?B signaling.
Carcinoma, Hepatocellular
Arginine deprivation, growth inhibition and tumour cell death: 3. Deficient utilisation of citrulline by malignant cells.
Carcinoma, Hepatocellular
Arginine depriving enzymes: applications as emerging therapeutics in cancer treatment.
Carcinoma, Hepatocellular
Argininosuccinate synthase 1 suppresses cancer cell invasion by inhibiting STAT3 pathway in hepatocellular carcinoma.
Carcinoma, Hepatocellular
Argininosuccinate synthase 1 suppresses tumor progression through activation of PERK/eIF2?/ATF4/CHOP axis in hepatocellular carcinoma.
Carcinoma, Hepatocellular
Combined lysosomal protein transmembrane 4 beta-35 and argininosuccinate synthetase expression predicts clinical outcome in hepatocellular carcinoma patients.
Carcinoma, Hepatocellular
Down-regulation of argininosuccinate synthetase is associated with cisplatin resistance in hepatocellular carcinoma cell lines: implications for PEGylated arginine deiminase combination therapy.
Carcinoma, Hepatocellular
Expression of argininosuccinate synthetase in patients with hepatocellular carcinoma.
Carcinoma, Hepatocellular
Expression profile and down-regulation of argininosuccinate synthetase in hepatocellular carcinoma in a transgenic mouse model.
Carcinoma, Hepatocellular
L-Canavanine potentiates the cytotoxicity of doxorubicin and cisplatin in arginine deprived human cancer cells.
Carcinoma, Hepatocellular
Pegylated recombinant human arginase (rhArg-peg5,000mw) inhibits the in vitro and in vivo proliferation of human hepatocellular carcinoma through arginine depletion.
Carcinoma, Hepatocellular
Phase II study of pegylated arginine deiminase for nonresectable and metastatic hepatocellular carcinoma.
Carcinoma, Hepatocellular
Reduced expression of ASS is closely related to clinicopathological features and post-resectional survival of hepatocellular carcinoma.
Carcinoma, Hepatocellular
Regulation of glucocorticoids of arginase and argininosuccinate synthetase in cultured rat hepatoma cells.
Carcinoma, Hepatocellular
Renal cell carcinoma does not express argininosuccinate synthetase and is highly sensitive to arginine deprivation via arginine deiminase.
Carcinoma, Hepatocellular
Targeted cellular metabolism for cancer chemotherapy with recombinant arginine-degrading enzymes.
Carcinoma, Hepatocellular
Targeting Arginine-Dependent Cancers with Arginine-Degrading Enzymes: Opportunities and Challenges.
Carcinoma, Hepatocellular
The Combination of Arginine Deprivation and 5-Fluorouracil Improves Therapeutic Efficacy in Argininosuccinate Synthetase Negative Hepatocellular Carcinoma.
Carcinoma, Neuroendocrine
Argininosuccinate synthetase (ASS) deficiency in high-grade pulmonary neuroendocrine carcinoma: an opportunity for personalized targeted therapy.
Carcinoma, Ovarian Epithelial
Aberrant regulation of argininosuccinate synthetase by TNF-alpha in human epithelial ovarian cancer.
Carcinoma, Renal Cell
Androgen receptor regulates ASS1P3/miR-34a-5p/ASS1 signaling to promote renal cell carcinoma cell growth.
Carcinoma, Renal Cell
Renal cell carcinoma does not express argininosuccinate synthetase and is highly sensitive to arginine deprivation via arginine deiminase.
Cataract
Urea cycle enzymes in retina, ciliary body-iris, lens and senile cataracts.
Cholangiocarcinoma
Decreased argininosuccinate synthetase expression in Thai patients with cholangiocarcinoma and the effects of ADI-PEG20 treatment in CCA cell lines.
Citrullinemia
A case of citrullinemia with abnormal messenger RNA for argininosuccinate synthetase.
Citrullinemia
A GC/MS-based metabolomic approach for diagnosing citrin deficiency.
Citrullinemia
A novel inborn error of metabolism detected by elevated methionine and/or galactose in newborn screening: neonatal intrahepatic cholestasis caused by citrin deficiency.
Citrullinemia
A novel Romani microdeletion variant in the promoter sequence of ASS1 causes citrullinemia type I.
Citrullinemia
A patient with type II citrullinemia who developed refractory complex seizure.
Citrullinemia
A search for the primary abnormality in adult-onset type II citrullinemia.
Citrullinemia
Additional mutations in argininosuccinate synthetase causing citrullinemia.
Citrullinemia
Ammonia Control in Children Ages 2 Months through 5 Years with Urea Cycle Disorders: Comparison of Sodium Phenylbutyrate and Glycerol Phenylbutyrate.
Citrullinemia
An adult-onset case of argininosuccinate synthetase deficiency presenting with atypical citrullinemia.
Citrullinemia
Analysis of a splice acceptor site mutation which produces multiple splicing abnormalities in the human argininosuccinate synthetase locus.
Citrullinemia
Analysis of deletions at the human argininosuccinate synthetase locus.
Citrullinemia
Application of mutation analysis for the previously uncertain cases of adult-onset type II citrullinemia (CTLN2) and their clinical profiles.
Citrullinemia
Argininosuccinate synthetase activity in cultured human lymphocytes.
Citrullinemia
Argininosuccinate synthetase deficiency and reye syndrome-like presentation.
Citrullinemia
Argininosuccinate synthetase deficiency: mutation analysis in 3 Thai patients.
Citrullinemia
Argininosuccinic acid synthetase deficiency in a hamster cell line and its complementation of argininosuccinic aciduria human fibroblasts.
Citrullinemia
Biochemical markers and neuropsychological functioning in distal urea cycle disorders.
Citrullinemia
Characterization of late-onset citrullinemia 1 in a Korean patient: confirmation by argininosuccinate synthetase gene mutation analysis.
Citrullinemia
Citrulline and Ammonia Accumulating in Citrullinemia Reduces Antioxidant Capacity of Rat Brain In Vitro.
Citrullinemia
Citrulline and ammonia accumulating in citrullinemia reduces antioxidant capacity of rat brain in vitro.
Citrullinemia
Citrullinemia presenting as uncontrollable epilepsy.
Citrullinemia
Citrullinemia Type 1: Behavioral Improvement with Late Liver Transplantation.
Citrullinemia
Citrullinemia type I and hypertrophic pyloric stenosis in a 1-month old male infant.
Citrullinemia
Citrullinemia type I is associated with a novel splicing variant, c.773 + 4A > C, in ASS1: a case report and literature review.
Citrullinemia
Citrullinemia Type I, Classical Variant. Identification of ASS approximately p.G390R (c.1168G>A) mutation in families of a limited geographic area of Argentina: A possible population cluster.
Citrullinemia
Citrullinemia with an Atypical Presentation: Paroxysmal Hypoventilation Attacks.
Citrullinemia
Citrullinemia with an atypical presentation: persistent hiccups. Case report.
Citrullinemia
Citrullinemia, argininosuccinate synthetase deficiency. Repository identification No. GM-1044.
Citrullinemia
Citrullinemia: enzymatic evidence for genetic heterogeneity.
Citrullinemia
Citrullinemia: quantitative deficiency of argininosuccinate synthetase in the liver.
Citrullinemia
Clearance of amino acids by hemodialysis in argininosuccinate synthetase deficiency.
Citrullinemia
Clinical and biochemical characteristics of patients with urea cycle disorders in a developing country.
Citrullinemia
Complete neurological recovery of an adult patient with type II citrullinemia after living related partial liver transplantation.
Citrullinemia
Concurrent triplication and uniparental isodisomy: evidence for microhomology-mediated break-induced replication model for genomic rearrangements.
Citrullinemia
Correction of argininosuccinate synthetase (AS) deficiency in a murine model of citrullinemia with recombinant adenovirus carrying human AS cDNA.
Citrullinemia
CT findings in the infantile form of citrullinemia.
Citrullinemia
Cytosolic Delivery of Argininosuccinate Synthetase Using a Cell-Permeant Miniature Protein.
Citrullinemia
Decreased plasma l-arginine levels in organic acidurias (MMA and PA) and decreased plasma branched-chain amino acid levels in urea cycle disorders as a potential cause of growth retardation: Options for treatment.
Citrullinemia
Detection of kinetically abnormal argininosuccinate synthase in neonatal citrullinemia by conversion of citrulline to arginine in intact fibroblasts.
Citrullinemia
Diagnosis and monitoring of inborn errors of metabolism using urease-pretreatment of urine, isotope dilution, and gas chromatography-mass spectrometry.
Citrullinemia
Effects of glucagon on urinary excretion of urea and on plasma ammonia level in argininosuccinate synthetase deficiency.
Citrullinemia
Electroencephalography and transcranial Doppler ultrasonography in neonatal citrullinemia.
Citrullinemia
Elevated plasma concentrations of the endogenous nitric oxide synthase inhibitor asymmetric dimethylarginine in citrullinemia.
Citrullinemia
Favorable long-term outcome following severe neonatal hyperammonemic coma in a patient with argininosuccinate synthetase deficiency.
Citrullinemia
First report of carglumic acid in a patient with citrullinemia type 1 (argininosuccinate synthetase deficiency).
Citrullinemia
Functional analysis of novel splicing and missense mutations identified in the ASS1 gene in classical citrullinemia patients.
Citrullinemia
General anesthesia in a patient with citrullinemia using Precedex as an adjunct to prevent delayed emergence.
Citrullinemia
Generation of a mouse model for citrullinemia by targeted disruption of the argininosuccinate synthetase gene.
Citrullinemia
Genetic approach to prenatal diagnosis in urea cycle defects.
Citrullinemia
Genomic structure of the adult-onset type II citrullinemia gene, SLC25A13, and cloning and expression of its mouse homologue.
Citrullinemia
Haemopoietic chimaerism: a complication in heterozygote detection tests for inherited defects in cattle.
Citrullinemia
Headache and neuropsychic disorders in the puerperium: a case report with suspected deficiency of urea cycle enzymes.
Citrullinemia
Hepatocellular carcinoma in a case of adult-onset type II citrullinemia.
Citrullinemia
Hepatocyte gene therapy in a large animal: a neonatal bovine model of citrullinemia.
Citrullinemia
Heterogeneity of mutations in argininosuccinate synthetase causing human citrullinemia.
Citrullinemia
Identification of 16 novel mutations in the argininosuccinate synthetase gene and genotype-phenotype correlation in 38 classical citrullinemia patients.
Citrullinemia
Identification of three novel mutations in fourteen patients with citrullinemia type 1.
Citrullinemia
Identification of two novel mutations in the SLC25A13 gene and detection of seven mutations in 102 patients with adult-onset type II citrullinemia.
Citrullinemia
Impaired T cell function in argininosuccinate synthetase deficiency.
Citrullinemia
Improved standards for prenatal diagnosis of citrullinemia.
Citrullinemia
Improving long term outcomes in urea cycle disorders-report from the Urea Cycle Disorders Consortium.
Citrullinemia
Improving the prenatal diagnosis of citrullinemia using citrulline/ornithine+arginine ratio in amniotic fluid.
Citrullinemia
In vivo urea cycle flux distinguishes and correlates with phenotypic severity in disorders of the urea cycle.
Citrullinemia
Incidence and distribution of argininosuccinate synthetase deficiency in human cancers: a method for identifying cancers sensitive to arginine deprivation.
Citrullinemia
Increased urinary excretion of argininosuccinate in type II citrullinemia.
Citrullinemia
Infantile cholestatic jaundice associated with adult-onset type II citrullinemia.
Citrullinemia
Investigation of citrullinemia type I variants by in vitro expression studies.
Citrullinemia
Kinetic mutations in argininosuccinate synthetase deficiency: characterisation and in vitro correction by substrate supplementation.
Citrullinemia
Letter: The high levels of lysine, homocitrulline, and homoarginine found in argininosuccinate synthetase deficiency.
Citrullinemia
Letter: The presence of the homoanalogues of substrates of the urea cycle in the presence of argininosuccinate synthetase deficiency.
Citrullinemia
Level of translatable messenger RNA coding for argininosuccinate synthetase in the liver of the patients with quantitative-type citrullinemia.
Citrullinemia
Liver transplantation as treatment for neurological disorders.
Citrullinemia
Liver transplantation may prevent neurodevelopmental deterioration in high-risk patients with urea cycle disorders.
Citrullinemia
Liver-directed adeno-associated virus serotype 8 gene transfer rescues a lethal murine model of citrullinemia type 1.
Citrullinemia
Living-related liver transplantation for type II citrullinemia using a graft from heterozygote donor.
Citrullinemia
Long-term outcome of urea cycle disorders: Report from a nationwide study in Japan.
Citrullinemia
Long-term survival of patients with argininosuccinate synthetase deficiency.
Citrullinemia
Messenger RNA coding for argininosuccinate synthetase in citrullinemia.
Citrullinemia
Metabolic derangements in deficiency of citrin, a liver-type mitochondrial aspartate-glutamate carrier.
Citrullinemia
Mild citrullinemia in Caucasians is an allelic variant of argininosuccinate synthetase deficiency (citrullinemia type 1).
Citrullinemia
Modelling urea-cycle disorder citrullinemia type 1 with disease-specific iPSCs.
Citrullinemia
Molecular analysis of argininosuccinate synthetase deficiency in human fibroblasts.
Citrullinemia
Molecular definition of bovine argininosuccinate synthetase deficiency.
Citrullinemia
Molecular epidemiology of citrullinemia type I in a risk region of Argentina: a first step to preconception heterozygote detection.
Citrullinemia
MRI in a case of adult-onset citrullinemia.
Citrullinemia
Mutation analysis of Korean patients with citrullinemia.
Citrullinemia
Mutations and DNA diagnoses of classical citrullinemia.
Citrullinemia
Mutations and polymorphisms in the human argininosuccinate synthetase (ASS1) gene.
Citrullinemia
Mutations in argininosuccinate synthetase mRNA in Japanese patients, causing classical citrullinemia.
Citrullinemia
Mutations in argininosuccinate synthetase mRNA of Japanese patients, causing classical citrullinemia.
Citrullinemia
Mutations in the Human Argininosuccinate Synthetase (ASS1) Gene, Impact on Patients, Common Changes, and Structural Considerations.
Citrullinemia
Nature and frequency of mutations in the argininosuccinate synthetase gene that cause classical citrullinemia.
Citrullinemia
Neonatal citrullinemia: novel, reversible neuroimaging findings correlated with ammonia level changes.
Citrullinemia
Neonatal presentation of adult-onset type II citrullinemia.
Citrullinemia
Neonatal type of argininosuccinate synthetase deficiency. Report of two cases with autopsy findings.
Citrullinemia
Orthotopic liver transplantation for urea cycle enzyme deficiency.
Citrullinemia
Pancreatic secretory trypsin inhibitor as a diagnostic marker for adult-onset type II citrullinemia.
Citrullinemia
Pancreatic secretory trypsin inhibitor gene is highly expressed in the liver of adult-onset type II citrullinemia.
Citrullinemia
Pathogenesis and pathophysiology of citrin (a mitochondrial aspartate glutamate carrier) deficiency.
Citrullinemia
Perinatal pathology casebook.
Citrullinemia
Physical, cognitive, and social status of patients with urea cycle disorders in Japan.
Citrullinemia
Plasma concentrations and renal clearance of orotic acid in argininosuccinic acid synthetase deficiency.
Citrullinemia
Postpartum "psychosis" in mild argininosuccinate synthetase deficiency.
Citrullinemia
Pregnancy in a healthy woman with untreated citrullinemia.
Citrullinemia
Prenatal diagnosis of citrullinemia and argininosuccinic aciduria: evidence for a transmission ratio distortion in citrullinemia.
Citrullinemia
Prenatal diagnosis of citrullinemia type 1: A Chinese family with a novel mutation of the ASS1 gene.
Citrullinemia
Prospective management of a child with neonatal citrullinemia.
Citrullinemia
Prospective treatment of urea cycle disorders.
Citrullinemia
Qualitative abnormality of liver argininosuccinate synthetase in a patient with citrullinemia.
Citrullinemia
Qualitative and quantitative abnormalities of argininosuccinate synthetase in citrullinemia.
Citrullinemia
Recurrent liver failure in a 25-year-old female.
Citrullinemia
Retroviral-mediated gene therapy for the treatment of citrullinemia. Transfer and expression of argininosuccinate synthetase in human hematopoietic cells.
Citrullinemia
Robust, Long-Term Culture of Endoderm-Derived Hepatic Organoids for Disease Modeling.
Citrullinemia
Role of liver transplantation in urea cycle disorders: Report from a nationwide study in Japan.
Citrullinemia
Saccharopinuria accompanied by hyperammonemia and hypercitrullinemia presented with elderly-onset epilepsy, progressive cognitive decline, and gait ataxia.
Citrullinemia
Slc25a13-knockout mice harbor metabolic deficits but fail to display hallmarks of adult-onset type II citrullinemia.
Citrullinemia
Structure of an abnormal messenger RNA for argininosuccinate synthetase in citrullinemia.
Citrullinemia
Structure of the human argininosuccinate synthetase gene and an improved system for molecular diagnostics in patients with classical and mild citrullinemia.
Citrullinemia
Studies on complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts.
Citrullinemia
Studies on liver argininosuccinate synthetase in a patient with citrullinemia and in normal subjects.
Citrullinemia
The Diagnosis and Management of a Female With Mild Citrullinemia and Undetectable Argininosuccinate Synthetase Activity in Fibroblasts.
Citrullinemia
The heterogeneous distribution of argininosuccinate synthetase in the liver of type II citrullinemic patients. Its specificity and possible clinical implications.
Citrullinemia
The human argininosuccinate synthetase locus and citrullinemia.
Citrullinemia
Toxoplasma gondii lacks the enzymes required for de novo arginine biosynthesis and arginine starvation triggers cyst formation.
Citrullinemia
Transiently reduced activity of carbamyl phosphate synthetase and ornithine transcarbamylase in liver of children with Reye's syndrome.
Citrullinemia
Two hypomorphic alleles of mouse Ass1 as a new animal model of citrullinemia type I and other hyperammonemic syndromes.
Citrullinemia
Type II (adult onset) citrullinaemia: clinical pictures and the therapeutic effect of liver transplantation.
Citrullinemia
Type II citrullinemia associated with neutropenia.
Citrullinemia
Type II citrullinemia in an elderly patient treated with living related partial liver transplantation.
Citrullinemia
Unrecognized citrullinemia mimicking encephalitis in a 14-year-old boy: unexpected result through the use of a standardized lumbar puncture protocol.
Citrullinemia
Urea cycle disorders in Argentine patients: clinical presentation, biochemical and genetic findings.
Citrullinemia
Urea cycle disorders in Thai infants: a report of 5 cases.
Citrullinemia
Usage of cryptic splice sites in citrullinemia fibroblasts suggests role of polyadenylation in splice-site selection during terminal exon definition.
Citrullinemia
Variation in argininosuccinate synthetase activity in amniotic fluid cell cultures: implications for prenatal diagnosis of citrullinemia.
Citrullinemia
[A case of citrullinemia with cluster type distribution of argininosuccinate synthetase in the liver]
Citrullinemia
[Adult-onset citrullinemia]
Citrullinemia
[Adult-onset type II citrullinemia in a patient undergoing continuous ambulatory peritoneal dialysis]
Citrullinemia
[Argininosuccinate synthetase deficiency]
Citrullinemia
[Citrullinemia type I with recurrent liver failure in a child].
Citrullinemia
[Recent advances of the treatment in metabolic disorders]
Citrullinemia
[Severe fulminant form of neonatal citrullinemia. Report of a case]
Citrullinemia
[The correlation of consciousness level and the concentration of CSF ammonia in a patient with adult-type citrullinemia]
Colonic Neoplasms
Arginine depriving enzymes: applications as emerging therapeutics in cancer treatment.
Colorectal Neoplasms
Argininosuccinate Synthase 1 is a Metabolic Regulator of Colorectal Cancer Pathogenicity.
Colorectal Neoplasms
Snail enhances arginine synthesis by inhibiting ubiquitination-mediated degradation of ASS1.
Coma
Favorable long-term outcome following severe neonatal hyperammonemic coma in a patient with argininosuccinate synthetase deficiency.
Coma
Liver transplantation for citrullinaemia improves intellectual function.
Coma
Prospective treatment of urea cycle disorders.
Costello Syndrome
Decreased elastin deposition and high proliferation of fibroblasts from Costello syndrome are related to functional deficiency in the 67-kD elastin-binding protein.
Costello Syndrome
The hyperthermia-enhanced association between tropoelastin and its 67-KDA chaperone results in better deposition of elastic fibers.
Cystic Fibrosis
Microarray analysis reveals the inhibition of intestinal expression of nutrient transporters in piglets infected with porcine epidemic diarrhea virus.
Dementia
Inducible nitric oxide synthase and argininosuccinate synthetase: co-induction in brain tissue of patients with Alzheimer's dementia and following stimulation with beta-amyloid 1-42 in vitro.
Dermatitis, Atopic
Neonatal gut colonization by Staphylococcus aureus strains with certain adhesins and superantigens is negatively associated with subsequent development of atopic eczema.
Dermatitis, Atopic
Superantigens and adhesins of infant gut commensal Staphylococcus aureus strains and association with subsequent development of atopic eczema.
Dystonia
Dystonia gene in Ashkenazi Jewish population is located on chromosome 9q32-34.
Dystonia
Identification of a highly polymorphic microsatellite VNTR within the argininosuccinate synthetase locus: exclusion of the dystonia gene on 9q32-34 as the cause of dopa-responsive dystonia in a large kindred.
Dystonia Musculorum Deformans
Linkage analysis in British and French families with idiopathic torsion dystonia.
Dystonia Musculorum Deformans
Linkage analysis with chromosome 9 markers in hereditary essential tremor.
Eczema
Superantigens and adhesins of infant gut commensal Staphylococcus aureus strains and association with subsequent development of atopic eczema.
Endometrial Neoplasms
Argininosuccinate Synthase 1-Deficiency Enhances the Cell Sensitivity to Arginine through Decreased DEPTOR Expression in Endometrial Cancer.
Epilepsy
Decreased glutamine synthetase, increased citrulline-nitric oxide cycle activities, and oxidative stress in different regions of brain in epilepsy rat model.
Fatty Liver
Long-chain fatty acids suppress the induction of urea cycle enzyme genes by glucocorticoid action.
Fatty Liver
Metabolic derangements in deficiency of citrin, a liver-type mitochondrial aspartate-glutamate carrier.
Fibrosarcoma
Cumulative influence of elastin peptides and plasminogen on matrix metalloproteinase activation and type I collagen invasion by HT-1080 fibrosarcoma cells.
fructose-bisphosphatase deficiency
Inherited metabolic disorders in Thailand.
Genetic Diseases, Inborn
Identification of three novel mutations in fourteen patients with citrullinemia type 1.
Genetic Diseases, Inborn
Retroviral-mediated gene therapy for the treatment of citrullinemia. Transfer and expression of argininosuccinate synthetase in human hematopoietic cells.
Genetic Diseases, Inborn
Usage of cryptic splice sites in citrullinemia fibroblasts suggests role of polyadenylation in splice-site selection during terminal exon definition.
Glioblastoma
Epigenetic status of argininosuccinate synthetase and argininosuccinate lyase modulates autophagy and cell death in glioblastoma.
Glioblastoma
Metabolomic profiling identifies distinct phenotypes for ASS1 positive and negative GBM.
Glioma
A Phase I study of pegylated arginine deiminase (pegargiminase), cisplatin and pemetrexed in argininosuccinate synthetase 1-deficient recurrent high-grade glioma.
Glioma
Presence of argininosuccinate synthetase in glial cells as revealed by peptide-specific antisera.
Hemangiosarcoma
Novel pathways and molecular targets for the treatment of sarcoma.
Hepatic Encephalopathy
Liver transplantation as treatment for neurological disorders.
Hepatic Encephalopathy
[Anesthetic management for a patient with citrullinemia and liver cirrhosis]
Hepatitis C, Chronic
[Serum argininosuccinate synthetase (ASS) in patients with chronic hepatitis C treated with interferon]
Herpes Simplex
Argininosuccinate synthetase 1 depletion produces a metabolic state conducive to herpes simplex virus 1 infection.
Homocystinuria
Inherited metabolic disorders in Thailand.
Hyperargininemia
Age at disease onset and peak ammonium level rather than interventional variables predict the neurological outcome in urea cycle disorders.
Hyperargininemia
Biochemical markers and neuropsychological functioning in distal urea cycle disorders.
Hyperargininemia
Genetic approach to prenatal diagnosis in urea cycle defects.
Hyperargininemia
Improving long term outcomes in urea cycle disorders-report from the Urea Cycle Disorders Consortium.
Hyperargininemia
Long-term outcome of urea cycle disorders: Report from a nationwide study in Japan.
Hyperglycinemia, Nonketotic
Clinical and genetic diversity of congenital hyperammonemia.
Hyperglycinemia, Nonketotic
Inherited metabolic disorders in Thailand.
Hyperlysinemias
Periodic hyperammonemia, hyperlysinemia, and homocitrullinuria associated with decreased argininosuccinate synthetase and arginase activities.
Hyperthyroidism
Triiodothyronine administration affects urea synthesis in rats.
Idiopathic Pulmonary Fibrosis
Therapeutic targeting of argininosuccinate synthase 1 (ASS1)-deficient pulmonary fibrosis.
Infections
Argininosuccinate synthetase 1 depletion produces a metabolic state conducive to herpes simplex virus 1 infection.
Infections
Oncogenic Kaposi's Sarcoma-Associated Herpesvirus Upregulates Argininosuccinate Synthase 1, a Rate-Limiting Enzyme of the Citrulline-Nitric Oxide Cycle, To Activate the STAT3 Pathway and Promote Growth Transformation.
Infections
Sustained generation of nitric oxide and control of mycobacterial infection requires argininosuccinate synthase 1.
Infections
Whole-Genome Sequencing Analysis of Methicillin-Resistant Staphylococcus simulans Causing Surgical Site Infection.
Insulin Resistance
Reversal of endothelial dysfunction in aorta of streptozotocin-nicotinamide-induced type-2 diabetic rats by S-Allylcysteine.
Insulinoma
Citrulline-argininosuccinate-arginine cycle coupled to Ca2+-signaling in rat pancreatic beta-cells.
Kidney Failure, Chronic
Effect of chronic renal failure on arginase and argininosuccinate synthetase expression.
Lethargy
Hyperammonemic Encephalopathy in an Adolescent Patient of Citrullinemia Type 1 With an Atypical Presentation.
Leukemia
Arginine depriving enzymes: applications as emerging therapeutics in cancer treatment.
Leukemia
Argininosuccinate synthetase gene expression in leukemias: potential diagnostic marker for blastic crisis of chronic myelocytic leukemia.
Leukemia
Elevated argininosuccinate synthetase activity in adult T leukemia cell lines.
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Argininosuccinate synthetase gene expression in leukemias: potential diagnostic marker for blastic crisis of chronic myelocytic leukemia.
Leukemia, Myeloid, Acute
NEI-01-induced arginine deprivation has potent activity against acute myeloid leukemia cells both in vitro and in vivo.
Liposarcoma
Novel pathways and molecular targets for the treatment of sarcoma.
Liver Diseases
Clearance of argininosuccinate synthetase from the circulation in acute liver disease.
Liver Diseases
Type II citrullinaemia (citrin deficiency) in a neonate with hypergalactosaemia detected by mass screening.
Liver Failure, Acute
A pregnant patient with fulminant hepatic failure was found to carry a novel missense mutation in the argininosuccinate synthetase gene.
Lung Diseases, Interstitial
Therapeutic targeting of argininosuccinate synthase 1 (ASS1)-deficient pulmonary fibrosis.
Lung Neoplasms
Arginine deiminase PEG20 inhibits growth of small cell lung cancers lacking expression of argininosuccinate synthetase.
Lung Neoplasms
Phase II Study of Arginine Deprivation Therapy With Pegargiminase in Patients With Relapsed Sensitive or Refractory Small-cell Lung Cancer.
Lung Neoplasms
The prognostic and therapeutic implications of distinct patterns of argininosuccinate synthase 1 (ASS1) and arginase-2 (ARG2) expression by cancer cells and tumor stroma in non-small-cell lung cancer.
Lupus Erythematosus, Systemic
Elevated gene expression of argininosuccinate synthetase in peripheral lymphocytes from systemic lupus erythematosus (SLE) patients.
Lymphoma
Targeting Arginine-Dependent Cancers with Arginine-Degrading Enzymes: Opportunities and Challenges.
Marfan Syndrome
Induction of macrophage chemotaxis by aortic extracts from patients with Marfan syndrome is related to elastin binding protein.
Melanoma
Activation of Ras/PI3K/ERK pathway induces c-Myc stabilization to upregulate argininosuccinate synthetase, leading to arginine deiminase resistance in melanoma cells.
Melanoma
Arginine deiminase augments the chemosensitivity of argininosuccinate synthetase-deficient pancreatic cancer cells to gemcitabine via inhibition of NF-?B signaling.
Melanoma
Arginine deprivation therapy for malignant melanoma.
Melanoma
Arginine deprivation, autophagy, apoptosis (AAA) for the treatment of melanoma.
Melanoma
Arginine deprivation, growth inhibition and tumour cell death: 3. Deficient utilisation of citrulline by malignant cells.
Melanoma
BRAF inhibitor resistance enhances vulnerability to arginine deprivation in melanoma.
Melanoma
Degradation of AMPK-?1 sensitizes BRAF inhibitor-resistant melanoma cells to arginine deprivation.
Melanoma
Deprivation of arginine by recombinant human arginase in prostate cancer cells.
Melanoma
Differential expression of argininosuccinate synthetase in serous and non-serous ovarian carcinomas.
Melanoma
Elastin-derived peptides upregulate matrix metalloproteinase-2-mediated melanoma cell invasion through elastin-binding protein.
Melanoma
High sensitivity of human melanoma cell lines to the growth inhibitory activity of mycoplasmal arginine deiminase in vitro.
Melanoma
Metabolic therapy with PEG-arginase induces a sustained complete remission in immunotherapy-resistant melanoma.
Melanoma
Negative argininosuccinate synthetase expression in melanoma tumours may predict clinical benefit from arginine-depleting therapy with pegylated arginine deiminase.
Melanoma
Pegylated arginine deiminase (ADI-SS PEG20,000 mw) inhibits human melanomas and hepatocellular carcinomas in vitro and in vivo.
Melanoma
Renal cell carcinoma does not express argininosuccinate synthetase and is highly sensitive to arginine deprivation via arginine deiminase.
Melanoma
Resistance to arginine deiminase treatment in melanoma cells is associated with induced argininosuccinate synthetase expression involving c-Myc/HIF-1alpha/Sp4.
Melanoma
Targeted cellular metabolism for cancer chemotherapy with recombinant arginine-degrading enzymes.
Melanoma
Targeting Arginine-Dependent Cancers with Arginine-Degrading Enzymes: Opportunities and Challenges.
Melanoma
Targeting argininosuccinate synthetase negative melanomas using combination of arginine degrading enzyme and cisplatin.
Melanoma
The relationship of arginine deprivation, argininosuccinate synthetase and cell death in melanoma.
Menkes Kinky Hair Syndrome
Inherited metabolic disorders in Thailand.
Mesothelioma
Arginine Deprivation With Pegylated Arginine Deiminase in Patients With Argininosuccinate Synthetase 1-Deficient Malignant Pleural Mesothelioma: A Randomized Clinical Trial.
Mesothelioma
Combination of arginine deprivation with TRAIL treatment as a targeted-therapy for mesothelioma.
Mesothelioma
In vivo loss of expression of argininosuccinate synthetase in malignant pleural mesothelioma is a biomarker for susceptibility to arginine depletion.
Mesothelioma
Metabolic response to pegylated arginine deiminase in mesothelioma with promoter methylation of argininosuccinate synthetase.
Mesothelioma, Malignant
Arginine Deprivation With Pegylated Arginine Deiminase in Patients With Argininosuccinate Synthetase 1-Deficient Malignant Pleural Mesothelioma: A Randomized Clinical Trial.
Mesothelioma, Malignant
Combination of arginine deprivation with TRAIL treatment as a targeted-therapy for mesothelioma.
Mesothelioma, Malignant
In vivo loss of expression of argininosuccinate synthetase in malignant pleural mesothelioma is a biomarker for susceptibility to arginine depletion.
Metabolism, Inborn Errors
Citrullinemia with an atypical presentation: persistent hiccups. Case report.
Mucopolysaccharidoses
Inherited metabolic disorders in Thailand.
Mucopolysaccharidosis I
Impaired elastogenesis in Hurler disease: dermatan sulfate accumulation linked to deficiency in elastin-binding protein and elastic fiber assembly.
Neoplasm Metastasis
Acid-Induced Downregulation of ASS1 Contributes to the Maintenance of Intracellular pH in Cancer.
Neoplasm Metastasis
Combined lysosomal protein transmembrane 4 beta-35 and argininosuccinate synthetase expression predicts clinical outcome in hepatocellular carcinoma patients.
Neoplasm Metastasis
Reduced argininosuccinate synthetase is a predictive biomarker for the development of pulmonary metastasis in patients with osteosarcoma.
Neoplasms
A cohort of supporting metabolic enzymes is coinduced with nitric oxide synthase in human tumor cell lines.
Neoplasms
A Phase II Study of Arginine Deiminase (ADI-PEG20) in Relapsed/Refractory or Poor-Risk Acute Myeloid Leukemia Patients.
Neoplasms
A proposed interaction mechanism between elastin-derived peptides and the elastin/laminin receptor-binding domain.
Neoplasms
Aberrant regulation of argininosuccinate synthetase by TNF-alpha in human epithelial ovarian cancer.
Neoplasms
Acid-Induced Downregulation of ASS1 Contributes to the Maintenance of Intracellular pH in Cancer.
Neoplasms
Activation of Ras/PI3K/ERK pathway induces c-Myc stabilization to upregulate argininosuccinate synthetase, leading to arginine deiminase resistance in melanoma cells.
Neoplasms
ADI, autophagy and apoptosis: metabolic stress as a therapeutic option for prostate cancer.
Neoplasms
Amino Acid Uptake Measured by [18F]AFETP Increases in Response to Arginine Starvation in ASS1-Deficient Sarcomas.
Neoplasms
Analysis of Gene Expression in 3D Spheroids Highlights a Survival Role for ASS1 in Mesothelioma.
Neoplasms
Androgen receptor regulates ASS1P3/miR-34a-5p/ASS1 signaling to promote renal cell carcinoma cell growth.
Neoplasms
Arginine deiminase augments the chemosensitivity of argininosuccinate synthetase-deficient pancreatic cancer cells to gemcitabine via inhibition of NF-?B signaling.
Neoplasms
Arginine deiminase PEG20 inhibits growth of small cell lung cancers lacking expression of argininosuccinate synthetase.
Neoplasms
Arginine dependence of tumor cells: targeting a chink in cancer's armor.
Neoplasms
Arginine deprivation and argininosuccinate synthetase expression in the treatment of cancer.
Neoplasms
Arginine deprivation as a strategy for cancer therapy: An insight into drug design and drug combination.
Neoplasms
Arginine deprivation as a targeted therapy for cancer.
Neoplasms
Arginine Deprivation Inhibits the Warburg Effect and Upregulates Glutamine Anaplerosis and Serine Biosynthesis in ASS1-Deficient Cancers.
Neoplasms
Arginine Deprivation With Pegylated Arginine Deiminase in Patients With Argininosuccinate Synthetase 1-Deficient Malignant Pleural Mesothelioma: A Randomized Clinical Trial.
Neoplasms
Arginine deprivation, growth inhibition and tumour cell death: 3. Deficient utilisation of citrulline by malignant cells.
Neoplasms
Arginine Starvation Impairs Mitochondrial Respiratory Function in ASS1-Deficient Breast Cancer Cells.
Neoplasms
Arginine starvation kills tumor cells through aspartate exhaustion and mitochondrial dysfunction.
Neoplasms
Arginine-Depleting Enzymes - An Increasingly Recognized Treatment Strategy for Therapy-Refractory Malignancies.
Neoplasms
Arginine: Challenges and opportunities of this two-faced molecule in cancer therapy.
Neoplasms
Argininosuccinate synthase 1 suppresses cancer cell invasion by inhibiting STAT3 pathway in hepatocellular carcinoma.
Neoplasms
Argininosuccinate synthase 1 suppresses tumor progression through activation of PERK/eIF2?/ATF4/CHOP axis in hepatocellular carcinoma.
Neoplasms
Argininosuccinate Synthetase 1 Loss in Invasive Bladder Cancer Regulates Survival through General Control Nonderepressible 2 Kinase-Mediated Eukaryotic Initiation Factor 2? Activity and Is Targetable by Pegylated Arginine Deiminase.
Neoplasms
Attenuation of argininosuccinate lyase inhibits cancer growth via cyclin A2 and nitric oxide.
Neoplasms
Autophagy maintains tumour growth through circulating arginine.
Neoplasms
Cationic Amino Acid Transporter-1-Mediated Arginine Uptake Is Essential for Chronic Lymphocytic Leukemia Cell Proliferation and Viability.
Neoplasms
Characterization of chromosome 9 deletions in transitional cell carcinoma by microsatellite assay.
Neoplasms
Chromatin accessibility governs the differential response of cancer and T cells to arginine starvation.
Neoplasms
Cisplatin-induced synthetic lethality to arginine-starvation therapy by transcriptional suppression of ASS1 is regulated by DEC1, HIF-1?, and c-Myc transcription network and is independent of ASS1 promoter DNA methylation.
Neoplasms
Co-application of canavanine and irradiation uncouples anticancer potential of arginine deprivation from citrulline availability.
Neoplasms
Collaboration Between RSK-EphA2 and Gas6-Axl RTK Signaling in Arginine Starvation Response That Confers Resistance to EGFR Inhibitors.
Neoplasms
Combination of arginine deprivation with TRAIL treatment as a targeted-therapy for mesothelioma.
Neoplasms
Combined lysosomal protein transmembrane 4 beta-35 and argininosuccinate synthetase expression predicts clinical outcome in hepatocellular carcinoma patients.
Neoplasms
Cytotoxicity of human recombinant arginase I (Co)-PEG5000 in the presence of supplemental L-citrulline is dependent on decreased argininosuccinate synthetase expression in human cells.
Neoplasms
Deprivation of arginine by recombinant human arginase in prostate cancer cells.
Neoplasms
Differential expression of argininosuccinate synthetase in serous and non-serous ovarian carcinomas.
Neoplasms
Down-regulation of argininosuccinate synthetase is associated with cisplatin resistance in hepatocellular carcinoma cell lines: implications for PEGylated arginine deiminase combination therapy.
Neoplasms
Endogenous arginase 2 as a potential biomarker for PEGylated arginase 1 treatment in xenograft models of squamous cell lung carcinoma.
Neoplasms
Engineering an arginine catabolizing bioconjugate: Biochemical and pharmacological characterization of PEGylated derivatives of arginine deiminase from Mycoplasma arthritidis.
Neoplasms
Gas6/Axl is the sensor of arginine-auxotrophic response in targeted chemotherapy with arginine-depleting agents.
Neoplasms
Histone deacetylase inhibition is synthetically lethal with arginine deprivation in pancreatic cancers with low argininosuccinate synthetase 1 expression.
Neoplasms
Imaging of tumor vascularization using fluorescence molecular tomography to monitor arginine deiminase treatment in melanoma.
Neoplasms
In-vivo evaluation of human recombinant Co-arginase against A375 melanoma xenografts.
Neoplasms
Incidence and distribution of argininosuccinate synthetase deficiency in human cancers: a method for identifying cancers sensitive to arginine deprivation.
Neoplasms
Increased expression of argininosuccinate synthetase protein predicts poor prognosis in human gastric cancer.
Neoplasms
Induction of citrulline-nitric oxide (NO) cycle enzymes and NO production in immunostimulated rat RPE-J cells.
Neoplasms
Induction of inducible nitric oxide synthase, argininosuccinate synthase, and GTP cyclohydrolase I in arthritic joints of human tumor necrosis factor-alpha transgenic mice.
Neoplasms
Intracellular arginine-dependent translation sensor reveals the dynamics of arginine starvation response and resistance in ASS1-negative cells.
Neoplasms
L-Canavanine Potentiates Cytotoxicity of Chemotherapeutic Drugs in Human Breast Cancer Cells.
Neoplasms
L-Canavanine potentiates the cytotoxicity of doxorubicin and cisplatin in arginine deprived human cancer cells.
Neoplasms
Metabolic therapy with PEG-arginase induces a sustained complete remission in immunotherapy-resistant melanoma.
Neoplasms
Molecular analysis of a myxoid chondrosarcoma with rearrangements of chromosomes 10 and 22.
Neoplasms
Naturally-occurring spinosyn A and its derivatives function as argininosuccinate synthase activator and tumor inhibitor.
Neoplasms
Negative argininosuccinate synthetase expression in melanoma tumours may predict clinical benefit from arginine-depleting therapy with pegylated arginine deiminase.
Neoplasms
Novel pathways and molecular targets for the treatment of sarcoma.
Neoplasms
Pegylated arginine deiminase (ADI-SS PEG20,000 mw) inhibits human melanomas and hepatocellular carcinomas in vitro and in vivo.
Neoplasms
Pegylated arginine deiminase: a novel anticancer enzyme agent.
Neoplasms
Phase 1 Dose-Escalation Study of Pegylated Arginine Deiminase, Cisplatin, and Pemetrexed in Patients With Argininosuccinate Synthetase 1-Deficient Thoracic Cancers.
Neoplasms
Phase 1 trial of ADI-PEG20 plus cisplatin in patients with pretreated metastatic melanoma or other advanced solid malignancies.
Neoplasms
Phase I/II study of pegylated arginine deiminase (ADI-PEG 20) in patients with advanced melanoma.
Neoplasms
Preclinical safety and antitumor activity of the arginine-degrading therapeutic enzyme pegzilarginase, a PEGylated, cobalt-substituted recombinant human arginase 1.
Neoplasms
Purification and immobilization of L-arginase from thermotolerant Penicillium chrysogenum KJ185377.1; with unique kinetic properties as thermostable anticancer enzyme.
Neoplasms
Recombinant human arginase inhibits proliferation of human hepatocellular carcinoma by inducing cell cycle arrest.
Neoplasms
Reduced expression of argininosuccinate synthetase 1 has a negative prognostic impact in patients with pancreatic ductal adenocarcinoma.
Neoplasms
RNA interference of argininosuccinate synthetase restores sensitivity to recombinant arginine deiminase (rADI) in resistant cancer cells.
Neoplasms
Selective extracellular arginine deprivation by a single injection of cellular non-uptake arginine deiminase nanocapsules for sustained tumor inhibition.
Neoplasms
Selective Intracellular Delivery of Recombinant Arginine Deiminase (ADI) Using pH-Sensitive Cell Penetrating Peptides To Overcome ADI Resistance in Hypoxic Breast Cancer Cells.
Neoplasms
State of the Art: Advances in Malignant Pleural Mesothelioma in 2017.
Neoplasms
Targeted cellular metabolism for cancer chemotherapy with recombinant arginine-degrading enzymes.
Neoplasms
Targeting Arginine-Dependent Cancers with Arginine-Degrading Enzymes: Opportunities and Challenges.
Neoplasms
Targeting argininosuccinate synthetase negative melanomas using combination of arginine degrading enzyme and cisplatin.
Neoplasms
The Combination of Arginine Deprivation and 5-Fluorouracil Improves Therapeutic Efficacy in Argininosuccinate Synthetase Negative Hepatocellular Carcinoma.
Neoplasms
The prognostic and therapeutic implications of distinct patterns of argininosuccinate synthase 1 (ASS1) and arginase-2 (ARG2) expression by cancer cells and tumor stroma in non-small-cell lung cancer.
Neoplasms
Tumor necrosis factor-alpha reduces argininosuccinate synthase expression and nitric oxide production in aortic endothelial cells.
Neoplasms
Unclassified hepatocellular adenoma expressing ASS1 associated with inflammatory hepatocellular adenomas.
Neurofibrosarcoma
Novel pathways and molecular targets for the treatment of sarcoma.
Neurologic Manifestations
Liver transplantation as treatment for neurological disorders.
ornithine carbamoyltransferase deficiency
Ammonia Control in Children Ages 2 Months through 5 Years with Urea Cycle Disorders: Comparison of Sodium Phenylbutyrate and Glycerol Phenylbutyrate.
ornithine carbamoyltransferase deficiency
Genetic approach to prenatal diagnosis in urea cycle defects.
ornithine carbamoyltransferase deficiency
Improving long term outcomes in urea cycle disorders-report from the Urea Cycle Disorders Consortium.
ornithine carbamoyltransferase deficiency
Liver transplantation may prevent neurodevelopmental deterioration in high-risk patients with urea cycle disorders.
ornithine carbamoyltransferase deficiency
Long-term outcome of urea cycle disorders: Report from a nationwide study in Japan.
ornithine carbamoyltransferase deficiency
New insights in nutritional management and amino acid supplementation in urea cycle disorders.
ornithine carbamoyltransferase deficiency
Physical, cognitive, and social status of patients with urea cycle disorders in Japan.
ornithine carbamoyltransferase deficiency
Role of liver transplantation in urea cycle disorders: Report from a nationwide study in Japan.
Ornithine Carbamoyltransferase Deficiency Disease
Ammonia Control in Children Ages 2 Months through 5 Years with Urea Cycle Disorders: Comparison of Sodium Phenylbutyrate and Glycerol Phenylbutyrate.
Ornithine Carbamoyltransferase Deficiency Disease
Genetic approach to prenatal diagnosis in urea cycle defects.
Ornithine Carbamoyltransferase Deficiency Disease
Improving long term outcomes in urea cycle disorders-report from the Urea Cycle Disorders Consortium.
Ornithine Carbamoyltransferase Deficiency Disease
Liver transplantation may prevent neurodevelopmental deterioration in high-risk patients with urea cycle disorders.
Ornithine Carbamoyltransferase Deficiency Disease
Long-term outcome of urea cycle disorders: Report from a nationwide study in Japan.
Ornithine Carbamoyltransferase Deficiency Disease
New insights in nutritional management and amino acid supplementation in urea cycle disorders.
Ornithine Carbamoyltransferase Deficiency Disease
Physical, cognitive, and social status of patients with urea cycle disorders in Japan.
Ornithine Carbamoyltransferase Deficiency Disease
Role of liver transplantation in urea cycle disorders: Report from a nationwide study in Japan.
Osteosarcoma
Reduced argininosuccinate synthetase is a predictive biomarker for the development of pulmonary metastasis in patients with osteosarcoma.
Ovarian Neoplasms
Cytotoxicity of [HuArgI (co)-PEG5000]-induced arginine deprivation to ovarian Cancer cells is autophagy dependent.
Ovarian Neoplasms
Differential expression of argininosuccinate synthetase in serous and non-serous ovarian carcinomas.
Ovarian Neoplasms
Epigenetic silencing of argininosuccinate synthetase confers resistance to platinum-induced cell death but collateral sensitivity to arginine auxotrophy in ovarian cancer.
Pancreatic Neoplasms
Histone deacetylase inhibition is synthetically lethal with arginine deprivation in pancreatic cancers with low argininosuccinate synthetase 1 expression.
Pancreatic Neoplasms
Pancreatic cancer cell lines deficient in argininosuccinate synthetase are sensitive to arginine deprivation by arginine deiminase.
pantoate-beta-alanine ligase (amp-forming) deficiency
Ammonia Control in Children Ages 2 Months through 5 Years with Urea Cycle Disorders: Comparison of Sodium Phenylbutyrate and Glycerol Phenylbutyrate.
pantoate-beta-alanine ligase (amp-forming) deficiency
Argininosuccinic acid synthetase deficiency in a hamster cell line and its complementation of argininosuccinic aciduria human fibroblasts.
pantoate-beta-alanine ligase (amp-forming) deficiency
Biochemical markers and neuropsychological functioning in distal urea cycle disorders.
pantoate-beta-alanine ligase (amp-forming) deficiency
Citrullinemia with an atypical presentation: persistent hiccups. Case report.
pantoate-beta-alanine ligase (amp-forming) deficiency
Orthotopic liver transplantation for urea cycle enzyme deficiency.
pantoate-beta-alanine ligase (amp-forming) deficiency
Plasma concentrations and renal clearance of orotic acid in argininosuccinic acid synthetase deficiency.
pantoate-beta-alanine ligase (amp-forming) deficiency
Prenatal diagnosis of citrullinemia and argininosuccinic aciduria: evidence for a transmission ratio distortion in citrullinemia.
pantoate-beta-alanine ligase (amp-forming) deficiency
Prospective treatment of urea cycle disorders.
pantoate-beta-alanine ligase (amp-forming) deficiency
Transiently reduced activity of carbamyl phosphate synthetase and ornithine transcarbamylase in liver of children with Reye's syndrome.
Phenylketonurias
Argininosuccinate synthetase gene is silenced by CpG methylation in children with phenylketonuria.
Phenylketonurias
Inherited metabolic disorders in Thailand.
Piebaldism
Second locus for Hirschsprung disease/Waardenburg syndrome in a large Mennonite kindred.
Propionic Acidemia
Inherited metabolic disorders in Thailand.
Prostatic Neoplasms
Arginine starvation-associated atypical cellular death involves mitochondrial dysfunction, nuclear DNA leakage, and chromatin autophagy.
Prostatic Neoplasms
Deprivation of arginine by recombinant human arginase in prostate cancer cells.
Pulmonary Fibrosis
Therapeutic targeting of argininosuccinate synthase 1 (ASS1)-deficient pulmonary fibrosis.
Retinoblastoma
Anti-tumor activity of arginine deiminase via arginine deprivation in retinoblastoma.
Rhabdomyosarcoma, Alveolar
Novel pathways and molecular targets for the treatment of sarcoma.
Sarcoma
Novel pathways and molecular targets for the treatment of sarcoma.
Sarcoma
Reduced argininosuccinate synthetase expression in refractory sarcomas: Impacts on therapeutic potential and drug resistance.
Sarcoma
Systems level profiling of arginine starvation reveals MYC and ERK adaptive metabolic reprogramming.
Sarcoma
Targeting Arginine-Dependent Cancers with Arginine-Degrading Enzymes: Opportunities and Challenges.
Sarcoma, Avian
Expression of human argininosuccinate synthetase after retroviral-mediated gene transfer.
Seizures
Hyperammonemic Encephalopathy in an Adolescent Patient of Citrullinemia Type 1 With an Atypical Presentation.
Sepsis
Hepatic glutamine metabolism in the septic rat.
Shock, Septic
Comparative genome analysis of global and Russian strains of community-acquired methicillin-resistant Staphylococcus aureus ST22, a 'Gaza clone'.
Solitary Fibrous Tumors
Novel pathways and molecular targets for the treatment of sarcoma.
Starvation
Metabolite regulation of argininosuccinate synthetase in cultured human cells.
Stomach Neoplasms
Argininosuccinate synthetase 1 contributes to gastric cancer invasion and progression by modulating autophagy.
Stomach Neoplasms
Argininosuccinate synthetase 1 suppression and arginine restriction inhibit cell migration in gastric cancer cell lines.
Stomach Neoplasms
Increased expression of argininosuccinate synthetase protein predicts poor prognosis in human gastric cancer.
Tremor
Linkage analysis with chromosome 9 markers in hereditary essential tremor.
Urea Cycle Disorders, Inborn
A novel Romani microdeletion variant in the promoter sequence of ASS1 causes citrullinemia type I.
Urea Cycle Disorders, Inborn
Citrullinemia Type 1: Behavioral Improvement with Late Liver Transplantation.
Urea Cycle Disorders, Inborn
Citrullinemia with an Atypical Presentation: Paroxysmal Hypoventilation Attacks.
Urea Cycle Disorders, Inborn
Clinical Outcomes of Neonatal Onset Proximal versus Distal Urea Cycle Disorders Do Not Differ.
Urea Cycle Disorders, Inborn
Improved standards for prenatal diagnosis of citrullinemia.
Urea Cycle Disorders, Inborn
Modelling urea-cycle disorder citrullinemia type 1 with disease-specific iPSCs.
Urea Cycle Disorders, Inborn
Mutations in the Human Argininosuccinate Synthetase (ASS1) Gene, Impact on Patients, Common Changes, and Structural Considerations.
Urea Cycle Disorders, Inborn
New insights in nutritional management and amino acid supplementation in urea cycle disorders.
Urea Cycle Disorders, Inborn
Robust, Long-Term Culture of Endoderm-Derived Hepatic Organoids for Disease Modeling.
Urea Cycle Disorders, Inborn
Steatogenesis in adult-onset type II citrullinemia is associated with down-regulation of PPAR?.
Urea Cycle Disorders, Inborn
Two hypomorphic alleles of mouse Ass1 as a new animal model of citrullinemia type I and other hyperammonemic syndromes.
Urea Cycle Disorders, Inborn
Unrecognized citrullinemia mimicking encephalitis in a 14-year-old boy: unexpected result through the use of a standardized lumbar puncture protocol.
Urinary Bladder Neoplasms
Argininosuccinate Synthetase 1 Loss in Invasive Bladder Cancer Regulates Survival through General Control Nonderepressible 2 Kinase-Mediated Eukaryotic Initiation Factor 2? Activity and Is Targetable by Pegylated Arginine Deiminase.
Urinary Bladder Neoplasms
Prognostic and therapeutic impact of argininosuccinate synthetase 1 control in bladder cancer as monitored longitudinally by PET imaging.
Urologic Neoplasms
Targeting Arginine-Dependent Cancers with Arginine-Degrading Enzymes: Opportunities and Challenges.
Uterine Cervical Neoplasms
Recombinant Bacillus caldovelox Arginase Mutant (BCA-M) Induces Apoptosis, Autophagy, Cell Cycle Arrest and Growth Inhibition in Human Cervical Cancer Cells.
Whooping Cough
Conformational dependence of collagenase (matrix metalloproteinase-1) up-regulation by elastin peptides in cultured fibroblasts.
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6.3.4.5 argininosuccinate synthase
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The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable). 
