• We show that argininosuccinate synthase 1 ( ASS1 ), a citrulline-aspartate ligase in de novo arginine synthesis pathway, was directly transactivated by p53 in response to genotoxic stress, resulting in the rearrangement of arginine metabolism. (sciencemag.org)
  • The ASS1 is an enzyme called argininosuccinate synthase 1. (creativebiomart.net)
  • The ASS1 gene provides instructions for making an enzyme called argininosuccinate synthase 1. (medlineplus.gov)
  • Diez-Fernandez C, Rüfenacht V, Häberle J. Mutations in the Human Argininosuccinate Synthetase (ASS1) Gene, Impact on Patients, Common Changes, and Structural Considerations. (medlineplus.gov)
  • An important gene associated with Citrullinemia, Classic is ASS1 (Argininosuccinate Synthase 1), and among its related pathways/superpathways are Arginine biosynthesis and Alanine, aspartate and glutamate metabolism . (malacards.org)
  • The team of scientists examined two types citrullinaemia ( amino acid disorders that do not allow cells to produce citrin--which is essential to removing urea, ammonia, and other toxic substances from the blood via the liver) to show that levels of Argininosuccinate synthase (ASS1), the missing enzyme that. (umd.edu)
  • Citrullinemia type I is an autosomal recessive disorder that is caused by a deficiency of the urea cycle enzyme argininosuccinate synthetase (ASS1). (ptglab.com)
  • Yuan, Yang;Mohammad, Mahmoud A;Betancourt, Ancizar;Didelija, Inka C;Yallampalli, Chandrasekar;Marini, Juan C 2018-06-07 00:00:00 Abstract Background The recycling of citrulline by argininosuccinate synthase 1 (ASS1) and argininosuccinate lyase (ASL) is crucial to maintain arginine availability and nitric oxide (NO) production. (deepdyve.com)
  • The availability of arginine seems to limit the synthesis of NO, because local arginase activity (5) or arginine supplementation (6) affects the rate of production of NO. Citrulline has the potential to be recycled intracellularly through the action of argininosuccinate synthase 1 (ASS1) and argininosuccinate lyase (ASL) to regenerate a new arginine molecule and thus sustain NO production (7, 8). (deepdyve.com)
  • But a multi-center phase 2 randomized clinical trial demonstrated that 68 of 201 mesothelioma tumors were deficient in argininosuccinate synthase (ASS1) , an enzyme necessary for arginine synthesis. (survivingmesothelioma.com)
  • Glucagon did not induce any of the urea cycle enzymes in adrenalectomized Sprague-Dawley rats and only induced argininosuccinate lyase (EC 4.3.2.1) in adrenalectomized inbred Wistar-Furth rats. (biomedsearch.com)
  • Arginine is synthesized from citrulline through two-step reactions catalyzed by AS and argininosuccinate lyase, in which AS is the rate-limiting enzyme. (aacrjournals.org)
  • Braissant O, Honegger P, Loup M, Iwase K, Takiguchi M, Bachmann C: Hyperammonemia: regulation of argininosuccinate synthetase and argininosuccinate lyase genes in aggregating cell cultures of fetal rat brain. (hmdb.ca)
  • In support of this hypothesis, the recycling enzymes, argininosuccinate synthase (AS) and argininosuccinate lyase (AL), have been shown to colocalize with eNOS in caveolae, a subcompartment of the plasma membrane. (biologists.org)
  • The synergistic effect was independent of basic and induced argininosuccinate synthase or argininosuccinate lyase protein expression and not abrogated by the presence of citrulline. (aacrjournals.org)
  • The kidney is the major organ for circulating L-Arg synthesis, utilizing citrulline conversion by argininosuccinate synthase (ASS) and lyase (ASL). (ufl.edu)
  • In the cytoplasm, L-ornithine is converted to L-arginine in three reactions mediated by ornithine carbamoyltransferase, arginosuccinate synthase, and argininosuccinate lyase. (wikipathways.org)
  • It is also said that a synthase is a lyase (a lyase is an enzyme that catalyzes the breaking of various chemical bonds by means other than hydrolysis and oxidation, often forming a new double bond or a new ring structure) and does not require any energy, whereas a synthetase is a ligase (a ligase is an enzyme that binds two chemicals or compounds) and thus requires energy. (wikipedia.org)
  • Zhang B, Cao GL, Domachowske J, Jackson MJ, Porasuphatana S, Rosen GM: Stable expression of varied levels of inducible nitric oxide synthase in primary cultures of endothelial cells. (hmdb.ca)
  • Zhang WY, Gotoh T, Oyadomari S, Mori M: Coinduction of inducible nitric oxide synthase and arginine recycling enzymes in cytokine-stimulated PC12 cells and high output production of nitric oxide. (hmdb.ca)
  • The enzyme endothelial nitric oxide synthase (eNOS) catalyzes the conversion of arginine, oxygen and NADPH to NO and citrulline. (biologists.org)
  • Endothelial nitric oxide synthase (eNOS), the enzyme that catalyzes the production of NO from the amino acid arginine in endothelial cells, plays a key role in vasoregulation as well as in other important physiological processes such as angiogenesis. (biologists.org)
  • Repeated induction of nitric oxide synthase and leishmanicidal activity in murine macrophages. (semanticscholar.org)
  • Murine macrophages express high levels of nitric oxide (NO) synthase and produce large amounts of NO when stimulated with interferon-gamma plus lipopolysaccharide in vitro. (semanticscholar.org)
  • The abnormal regulation of inducible nitric oxide synthase (iNOS) and neuronal nitric oxide synthase (nNOS) is associated with neurodegenerative disorders. (hindawi.com)
  • Nitric oxide (NO) is important for many synthases (NOSs, EC 1.14.13.39), including inducible nitric oxide synthase (iNOS), neuronal nitric oxide synthase (nNOS), and endothelial nitric oxide synthase (eNOS) [ 1 ]. (hindawi.com)
  • NOS, nitric oxide synthase. (deepdyve.com)
  • This radiosensitization of the tumor cells was inhibited by a nitric oxide (NO) synthase inhibitor so they realized that this sensitization was due to NO stimulation. (denvernaturopathic.com)
  • Nitric oxide recycling is an important function that can sometimes be inadequate in those with the B antigen (blood groups B and AB), probably due to genetic linkage of the argininosuccinate synthase enzyme. (dadamo.com)
  • Biochemical nomenclature has sometimes distinguished synthetases from synthases and sometimes treated the words as synonyms. (wikipedia.org)
  • Increased metabolism of l -Arginine ( l -Arg), through the enzymes arginase 1 and NO synthase 2 (NOS2), is well documented as a major MDSC suppressive mechanism. (jimmunol.org)
  • Pharmaceutical grade L-citrulline supplements are used for carbamoylphosphate synthetase (CPS) and ornithine transcarbamylase (OTC) deficiency and L-arginine is used in case of argininosuccinate synthase deficiency and citrullinemia to catalyze the enzymes in urea cycle and support optimum ammonia removal. (news-medical.net)
  • Importantly, lack of ASS also influences essential enzymes in pyrimidine synthesis (carbamoyl-phosphate synthetase2, aspartate transcarbamylase and dihydrooratase (CAD) and thymidylate synthase (TS)) and malate dehydrogenase-1 (MDH-1) in TCA cycle. (elsevier.com)
  • Increased expression and activities of arginase 1 (ARG1) and NO synthase 2 (NOS2) are well established as the hallmarks for MDSC suppressive function ( 11 ). (jimmunol.org)
  • Sequences of the genes encoding argininosuccinate synthetase in Escherichia coli and Saccharomyces cerevisiae: comparison with methanogenic archaebacteria and mammals. (ebi.ac.uk)
  • Conservation of structure in the human gene encoding argininosuccinate synthetase and the argG genes of the archaebacteria Methanosarcina barkeri MS and Methanococcus vannielii. (ebi.ac.uk)
  • Argininosuccinate Synthase 1-Deficiency Enhances the Cell Sensitivity to Arginine through Decreased DEPTOR Expression in Endometrial Cancer. (abcam.com)
  • We studied the possible mechanisms of NO deficiency in various models of CKD, focusing on possible reduced NO synthase (NOS) substrate (L-arginine, L-Arg). (ufl.edu)
  • Most of the mutations involved in type I citrullinemia change single amino acids in the argininosuccinate synthase 1 enzyme. (medlineplus.gov)
  • Structure of the human argininosuccinate synthetase gene and an improved system for molecular diagnostics in patients with classical and mild citrullinemia. (medlineplus.gov)
  • In the context of a proteomic screening of intestinal modifications induced by dietary spermine in suckling rats, we showed that argininosuccinate synthetase and carbamoyl phosphate synthase disappeared from enterocytes after this treatment. (ac.be)
  • Various tumor types have been shown to lack expression of the enzyme argininosuccinate synthase (ASS) that is required for the synthesis of arginine and thus rely on exogenous sources for protein synthesis requirements. (aacrjournals.org)
  • Four proteins, namely, heat shock 70 kDa protein 1, argininosuccinate synthase, isoform 2 of UTP-glucose-1-phosphate uridylyltransferase, and transketolase, were shown to have the potential to differentiate metastatic relapse (MR) from nonrelapse (NR) HCC patients after validation by western blotting and immunohistochemical assays. (sigmaaldrich.com)
  • Argininosuccinate synthase ( EC:6.3.4.5 ) (AS) is a urea cycle enzyme that catalyzes the penultimate step in arginine biosynthesis: the ATP-dependent ligation of citrulline to aspartate to form argininosuccinate, AMP and pyrophosphate [ PMID: 2123815 , PMID: 3133361 ]. (ebi.ac.uk)
  • 1.73 Angstrom Resolution Crystal Structure of Dihydropteroate Synthase (folP-SMZ_B27) from Soil Uncultured Bacterium. (csgid.org)
  • This entry represents argininosuccinate synthase, type 2 subfamily. (ebi.ac.uk)
  • The down-regulated proteins, notably six heat shock proteins and two ATP synthases, have important roles in the mobilization of carbohydrates and energy, and in the balance between synthesis and degradation of other proteins during seed deterioration. (frontiersin.org)
  • ADI-PEG20, arginine, argininosuccinate, citrulline, mouse, recycling Introduction Arginine is a conditionally indispensable amino acid that serves as a precursor for the synthesis of numerous compounds. (deepdyve.com)
  • The synthesis of NO is catalyzed by the oxidation of the imino group of arginine by intracellular NO synthase (NOS), generating citrulline as a coproduct of this reaction (4) (Figure 1). (deepdyve.com)
  • However, the Joint Commission on Biochemical Nomenclature (JCBN) dictates that "synthase" can be used with any enzyme that catalyses synthesis (whether or not it uses nucleoside triphosphates), whereas "synthetase" is to be used synonymously. (wikipedia.org)
  • Loss of argininosuccinate synthetase (ASS) expression in melanoma makes these tumor cells vulnerable to arginine deprivation. (elsevier.com)
  • The up-regulation of argininosuccinate synthase participated in proline biosynthesis at 16% MC, which is important for maintaining reactive oxygen species homeostasis for the resistance of heat stress. (frontiersin.org)
  • The sensitivity of melanoma to ADI treatment is based on its auxotrophy for arginine due to a lack of argininosuccinate synthetase (AS) expression, the rate-limiting enzyme for the de novo biosynthesis of arginine. (aacrjournals.org)
  • Tresch S, Heilmann M, Christiansen N, Looser R, Grossmann K. Inhibition of saturated very-long-chain fatty acid biosynthesis by mefluidide and perfluidone, selective inhibitors of 3-ketoacyl-CoA synthases. (labome.org)
  • Argininosuccinate synthetase (ASS), a key enzyme to synthesize arginine is down regulated in many tumors including hepatocellular carcinoma (HCC). (elsevier.com)
  • Although the importance of both intestinal and renal argininosuccinate synthetases has been recognised for a long time, nutrients have not yet been identified as inducers of the gene expression. (ac.be)
  • Argentilactone inhibited the enzymatic activity of malate dehydrogenase, citrate synthase, and pyruvate dehydrogenase. (asm.org)
  • Defects in argininosuccinate synthase 1 disrupt the third step of the urea cycle, preventing the liver from processing excess nitrogen into urea. (medlineplus.gov)
  • Argininosuccinate synthetase, an ubiquitous enzyme in mammals, catalyses the formation of argininosuccinate, the precursor of arginine. (ac.be)
  • Genetic Manipulation of Leishmania donovani to Explore the Involvement of Argininosuccinate Synthase in Oxidative Stress Management. (semanticscholar.org)
  • It has been reported that majority of melanomas and hepatocellular carcinomas are auxotrophic for arginine because of their lack of argininosuccinate synthetase (AS) expression ( 3 - 5 ). (aacrjournals.org)
  • The expression of NO synthase peaks at 12 h after stimulation and declines rapidly to the background level by 72 h. (semanticscholar.org)
  • Following MDI aerosol exposure, expression of a number of proteins with immunological or xenobiotic metabolism relevance is increased, including endoplasmin, cytochrome P450 and argininosuccinate synthase. (cdc.gov)
  • Belongs to the argininosuccinate synthase family. (abcam.com)