Argininosuccinate Synthase (Argininosuccinate Synthetase)

An enzyme of the urea cycle that catalyzes the formation of argininosuccinic acid from citrulline and aspartic acid in the presence of ATP. Absence or deficiency of this enzyme causes the metabolic disease CITRULLINEMIA in humans. EC 6.3.4.5.

Also Known As:

Networked: 188 relevant articles (5 outcomes, 8 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Savaraj, Niramol: 7 articles (03/2015 - 01/2007)
2.	Szlosarek, Peter W: 7 articles (02/2014 - 12/2006)
3.	Kung, Hsing-Jien: 6 articles (09/2014 - 10/2008)
4.	Saheki, Takeyori: 6 articles (10/2005 - 12/2002)
5.	Kobayashi, Keiko: 6 articles (10/2005 - 12/2002)
6.	Bomalaski, John S: 5 articles (08/2015 - 10/2002)
7.	Feun, Lynn G: 5 articles (03/2015 - 08/2010)
8.	Kuo, Macus Tien: 5 articles (03/2015 - 12/2009)
9.	Bold, Richard J: 5 articles (09/2014 - 10/2008)
10.	Frankel, Arthur E: 4 articles (12/2014 - 01/2012)

Related Diseases

1.	Citrullinemia 04/01/2002 - "Structure of the human argininosuccinate synthetase gene and an improved system for molecular diagnostics in patients with classical and mild citrullinemia." 03/01/2005 - "Previous studies showed short-term correction of bovine and murine argininosuccinate synthetase (ASS) deficiency after first generation adenoviral-mediated liver gene therapy. " 01/01/1979 - "Studies on liver argininosuccinate synthetase in a patient with citrullinemia and in normal subjects." 01/01/2015 - "Case 1 has a triplication at 9q21.11-q21.33 and segmental paternal isoUPD for 9q21.33-qter, and presented with citrullinemia with a homozygous mutation in the argininosuccinate synthetase gene (ASS1 at 9q34.1). " 11/01/2014 - "The authors present a case of citrullinemia with a genotype of argininosuccinate synthetase (ASS1), c.380 G>A (p.R127Q)/c.380 G>A (p.R127Q), in two alleles. "
2.	Neoplasms (Cancer) 01/01/2015 - "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. " 12/01/2014 - "Unfortunately, after 1 month, half of the relapsing tumors showed argininosuccinate synthase induction, which correlated with Ser62-phosphorylated cMyc. " 11/01/2006 - "Both bioconjugates exhibited potent growth inhibition of several cultured tumor lines that are deficient in the anabolic enzyme, argininosuccinate synthetase. " 01/01/2015 - "Aberrant expression of argininosuccinate synthetase (ASS1, also known as ASS) has been found in cancer cells and is involved in the carcinogenesis of gastric cancer. " 04/01/2014 - "Argininosuccinate synthetase 1 (ASS1) is a key enzyme in arginine biosynthesis, and its abundance is reduced in many solid tumors, making them sensitive to external arginine depletion. "
3.	Melanoma (Melanoma, Malignant) 09/01/1992 - "Five melanoma cell lines tested also exhibited reduced argininosuccinate synthetase (ASS) gene expression--being almost absent in four cell lines and at low level in one cell line. " 01/01/2013 - "Melanoma cells frequently lack argininosuccinate synthetase, an essential enzyme for arginine synthesis, and as a result they become dependent on the availability of exogenous arginine. " 05/15/2012 - "Activation of Ras/PI3K/ERK pathway induces c-Myc stabilization to upregulate argininosuccinate synthetase, leading to arginine deiminase resistance in melanoma cells." 04/24/2012 - "Advanced melanoma patients with accessible tumours had biopsy performed before the start of treatment with ADI-PEG20 and at the time of progression or relapse when amenable to determine whether argininosuccinate synthetase (ASS) expression in tumour was predictive of response to ADI-PEG20. " 12/01/2009 - "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. "
4.	Liver Diseases (Liver Disease) 04/01/1990 - "Clearance of argininosuccinate synthetase from the circulation in acute liver disease." 02/01/2002 - "Type II citrullinaemia (CTLN2) is an adult- or late childhood-onset liver disease characterized by a liver-specific defect in argininosuccinate synthetase protein. "
5.	Hepatocellular Carcinoma (Hepatoma) 01/01/2015 - "Expression profile and down-regulation of argininosuccinate synthetase in hepatocellular carcinoma in a transgenic mouse model." 02/01/2013 - "Expression of argininosuccinate synthetase in patients with hepatocellular carcinoma." 06/01/2011 - "Combined lysosomal protein transmembrane 4 beta-35 and argininosuccinate synthetase expression predicts clinical outcome in hepatocellular carcinoma patients." 09/28/2010 - "Human hepatocellular carcinoma (HCC) cells are largely deficient of argininosuccinate synthetase and thus auxotrophic for arginine. " 05/01/2010 - "It is well known that hepatocellular carcinoma (HCC) is an arginine auxotroph due to argininosuccinate synthetase I deficiency. "

Related Drugs and Biologics

1.	Urea (Carbamide)
2.	Arginine (L-Arginine)
3.	ADI PEG20
4.	Citrulline
5.	Argininosuccinate Lyase
6.	Protein Isoforms (Isoforms)
7.	Superoxide Dismutase
8.	arginine deiminase
9.	Messenger RNA (mRNA)
10.	Arginase

Related Therapies and Procedures

1.	Drug Therapy (Chemotherapy)
2.	Oral Administration