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Argininosuccinate synthase 1

Argininosuccinate Synthase, argininosuccinate synthetase
The protein encoded by this gene catalyzes the penultimate step of the arginine biosynthetic pathway. There are approximately 10 to 14 copies of this gene including the pseudogenes scattered across the human genome, among which the one located on chromosome 9 appears to be the only functional gene for argininosuccinate synthetase. Mutations in the chromosome 9 copy of ASS cause citrullinemia. Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: ACID, Arginase, CAN, HAD, iNOS
Papers on Argininosuccinate Synthase
BRAF inhibitor resistance enhances vulnerability to arginine deprivation in melanoma.
Savaraj et al., Miami, United States. In Oncotarget, Feb 2016
Here, we demonstrate that BRAFi-resistant (BR) melanomas are susceptible to arginine deprivation due to inability to initiate re-expression of argininosuccinate synthetase (ASS1, a key enzyme for arginine synthesis) as well as ineffective autophagy.
Selective Intracellular Delivery of Recombinant Arginine Deiminase (ADI) Using pH-Sensitive Cell Penetrating Peptides To Overcome ADI Resistance in Hypoxic Breast Cancer Cells.
Shen et al., Taipei, Taiwan. In Mol Pharm, Feb 2016
Many tumor cells have suppressed expression of a key enzyme, argininosuccinate synthetase 1 (ASS1), which converts citrulline to arginine.
Controlling the transcription levels of argGH redistributed L-arginine metabolic flux in N-acetylglutamate kinase and ArgR-deregulated Corynebacterium crenatum.
Xu et al., Wuxi, China. In J Ind Microbiol Biotechnol, Jan 2016
Genetic and enzymatic levels analysis involved in L-arginine biosynthetic pathway of recombinants SYPA5-5-NAGKH268N (H-7) and SYPA5-5-NAGKR209A (R-8) showed that the transcription levels of argGH decreased accompanied with the reduction of argininosuccinate synthase and argininosuccinase activities, respectively, which led to the metabolic obstacle from L-citrulline to L-arginine.
Ferulic acid enhances nitric oxide production through up-regulation of argininosuccinate synthase in inflammatory human endothelial cells.
Matsui et al., Fukuoka, Japan. In Life Sci, Jan 2016
N(G)-monomethyl-l-arginine acetate (a nitric oxide synthase (NOS) inhibitor) and α-methyl-dl-aspartic acid (an argininosuccinate synthase (ASS) inhibitor) counteracted the effects of FA on the NO production.
Neonatal mortality and outcome at the end of the first year of life in early onset urea cycle disorders-review and meta-analysis of observational studies published over more than 35 years.
Hoffmann et al., Heidelberg, Germany. In J Inherit Metab Dis, Jan 2016
Random effects meta-analysis was done for four UCDs, deficiency of carbamylphosphate synthetase 1 (CPS1D), male/female ornithine transcarbamylase (OTCDm/f), argininosuccinate synthetase (ASSD) and lyase (ASLD).
Arginine deprivation in cancer therapy.
Savaraj et al., Miami, United States. In Curr Opin Clin Nutr Metab Care, 2015
RECENT FINDINGS: New developments in this area include understanding of the role of most significantly downregulated gene regulating amino acid metabolism, argininosuccinate synthetase and its expression and therapeutic relevance in different tumors.
Diagnosis and treatment of urea cycle disorder in Japan.
Endo et al., Kumamoto, Japan. In Pediatr Int, 2014
Decreased excretion of nitrogen in the urea cycle due to deficiency of carbamoyl phosphate synthase I (CPSI), ornithine transcarbamylase (OTC), argininosuccinate synthase (ASS), argininosuccinate lyase (ASL), and N-acetyl glutamate synthase (NAGS) causes hyperammonemia.
Targeting arginine-dependent cancers with arginine-degrading enzymes: opportunities and challenges.
Szlosarek et al., London, United Kingdom. In Cancer Res Treat, 2013
Several studies have focused on inactivation of argininosuccinate synthetase 1 (ASS1) in a range of malignancies, including melanoma, hepatocellular carcinoma (HCC), mesothelial and urological cancers, sarcomas, and lymphomas.
Regulation of endothelial nitric oxide synthase activity by protein-protein interaction.
Su, Augusta, United States. In Curr Pharm Des, 2013
In addition, eNOS associations with cationic amino acid transporter-1 (CAT-1), argininosuccinate synthase (ASS), argininosuccinate lyase (ASL), and soluble guanylate cyclase (sGC) facilitate directed delivery of substrate (L-arginine) to eNOS and optimizing NO production and NO action on its target.
Novel pathways and molecular targets for the treatment of sarcoma.
Van Tine et al., Saint Louis, United States. In Curr Oncol Rep, 2013
Recently identified novel druggable targets include the MDM2 amplifications in well-differentiated and dedifferentiated liposarcomas, the new translocation NAB2:STAT6 of solitary fibrous tumors, the angiopoeitin-TIE2 pathway in angiosarcoma, the suppression of Mcl1 in X:18/synovial sarcomas, the mTOR pathway in malignant peripheral nerve sheath tumors, CDK4 in alveolar rhabdomyosarcoma, cMET regulation in alveolar soft parts sarcoma, the metabolic abnormalities in wild-type/SHD GIST, and the lack of argininosuccinate synthetase 1 expression seen in most sarcomas.
Argininosuccinate synthase conditions the response to acute and chronic ethanol-induced liver injury in mice.
Nieto et al., New York City, United States. In Hepatology, 2012
Partial argininosuccinate synthase ablation protects only in acute ethanol-induced liver injury by decreasing nitrosative stress but not in a more chronic scenario where oxidative stress and impaired fatty acid beta-oxidation are key events.
A regulatory role of Kruppel-like factor 4 in endothelial argininosuccinate synthetase 1 expression in response to laminar shear stress.
Boo et al., Taegu, South Korea. In Biochem Biophys Res Commun, 2012
The present study demonstrated a key regulatory role of KLF4 in the endothelial ASS1 expression and NO production in response to laminar shear stress.
Inhibition of LPS toxicity by hepatic argininosuccinate synthase (ASS): novel roles for ASS in innate immune responses to bacterial infection.
Svetlov et al., United States. In Int Immunopharmacol, 2011
The ASS release represents a potential counteracting liver reaction to LPS.
Endothelial argininosuccinate synthetase 1 regulates nitric oxide production and monocyte adhesion under static and laminar shear stress conditions.
Boo et al., Taegu, South Korea. In J Biol Chem, 2011
the expression of ASS1 harmonized with that of NOS3 may be important for the optimized endothelial NO production and the prevention of the inflammatory monocyte adhesion to endothelial cells.
Polymorphic variants of genes related to arginine metabolism and the risk of orofacial clefts.
Jagodzinski et al., Warsaw, Poland. In Arch Oral Biol, 2010
Analysis of five SNPs of the ASS1 gene revealed that the G allele of rs7860909 is associated with increased CL/P risk.
Phase II study of pegylated arginine deiminase for nonresectable and metastatic hepatocellular carcinoma.
Izzo et al., Houston, United States. In J Clin Oncol, 2010
PURPOSE: It is well known that hepatocellular carcinoma (HCC) is an arginine auxotroph due to argininosuccinate synthetase I deficiency.
The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein.
Saheki et al., Kagoshima, Japan. In Nat Genet, 1999
Citrullinaemia (CTLN) is an autosomal recessive disease caused by deficiency of argininosuccinate synthetase (ASS).
Abnormal mRNA for argininosuccinate synthetase in citrullinaemia.
O'Brien et al., In Nature, 1983
Citrullinaemia is a human inborn error of metabolism resulting from the deficiency of argininosuccinate synthetase.
Dispersion of argininosuccinate-synthetase-like human genes to multiple autosomes and the X chromosome.
Ruddle et al., In Cell, 1982
DNA sequences closely homologous to argininosuccinate synthetase are present at ten or more distinct locations in the human genome, including sites on chromosomes 6, 9 and X. Argininosuccinate synthetase thus represents one of the most widely dispersed multigene families described to date, the first instance of a multigene family associated with an enzyme of intermediary metabolism and, perhaps most striking, the first instance of a multigene family with members on both autosomes and sex chromosomes.
Ornithine-Urea Cycle Enzymes in the African Lungfish, Protopterus aethiopicus.
Cohen et al., In Science, 1966
Levels of activity of the rate-limiting enzymes, carbamoyl phosphate synthetase and argininosuccinate synthetase, are similar to those in the premetamorphic tadpole of Rana catesbeiana and considerably lower than the levels reported for other ureotelic animals.
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