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: 321 relevant articles (9 outcomes, 20 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Savaraj, Niramol: 13 articles (02/2020 - 01/2007)
2.	Bomalaski, John S: 12 articles (01/2021 - 10/2002)
3.	Szlosarek, Peter W: 12 articles (11/2020 - 12/2006)
4.	Feun, Lynn G: 10 articles (02/2020 - 08/2010)
5.	Johnston, Amanda: 8 articles (01/2022 - 01/2017)
6.	Kung, Hsing-Jien: 8 articles (01/2021 - 10/2008)
7.	Kuo, Macus Tien: 8 articles (02/2020 - 12/2009)
8.	Wangpaichitr, Medhi: 7 articles (12/2017 - 01/2007)
9.	Häberle, Johannes: 7 articles (08/2017 - 11/2003)
10.	Bomalaski, John: 6 articles (01/2022 - 03/2015)

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." 12/14/2023 - "Citrullinemia type I is a rare autosomal-recessive disorder caused by deficiency of argininosuccinate synthetase (ASS1). " 01/01/2023 - "Citrullinemia type 1 (CTLN1) is a rare autosomal recessive disease caused by argininosuccinate synthetase (ASS) deficiency. "
2.	Neoplasms (Cancer) 01/01/2021 - "We enrolled 99 patients with metastatic argininosuccinate synthetase 1 (ASS1) deficient malignancies. " 01/01/2020 - "Argininosuccinate synthase (ASS1) downregulation in different tumors has been shown to support cell proliferation and yet, in several common cancer subsets ASS1 expression associates with poor patient prognosis. " 10/01/2019 - "The third 6 cm nodule had all the characteristics of an Unclassified HCA with an overexpression of Argininosuccinate Synthase 1 (ASS1) in the tumor compared to the non-tumoral liver. " 12/01/2014 - "Unfortunately, after 1 month, half of the relapsing tumors showed argininosuccinate synthase induction, which correlated with Ser62-phosphorylated cMyc. " 08/03/2021 - "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."
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. " 12/01/2017 - "It has been reported that naïve melanoma cells undergo autophagy and re-express argininosuccinate synthetase 1 (ASS1) to enable them to synthesize arginine for survival when encountering arginine deprivation. " 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 - "Resistance to arginine deiminase treatment in melanoma cells is associated with induced argininosuccinate synthetase expression involving c-Myc/HIF-1alpha/Sp4."
4.	Hepatocellular Carcinoma (Hepatoma) 01/23/2015 - "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." 01/01/2010 - "Argininosuccinate synthetase (ASS) has previously been proven to be reductively expressed in hepatocellular carcinoma (HCC) and various types of HCC cell lines. " 04/10/2021 - "Argininosuccinate synthase 1 suppresses tumor progression through activation of PERK/eIF2α/ATF4/CHOP axis in hepatocellular carcinoma." 03/01/2019 - "Argininosuccinate synthase 1 suppresses cancer cell invasion by inhibiting STAT3 pathway in hepatocellular carcinoma."
5.	Small Cell Lung Carcinoma (Small Cell Lung Cancer) 01/17/2012 - "Argininosuccinate synthetase expression was assessed in human small cell lung cancer (SCLC) by immunohistochemistry (IHC), with expression also assessed in a panel of 10 human SCLC by qRT-PCR and western blot. " 11/01/2020 - "Pre-clinical studies indicated that arginine-deprivation therapy using pegylated arginine deiminase (pegargiminase, ADI-PEG 20) may be effective in patients with argininosuccinate synthetase 1 (ASS1)-deficient small-cell lung cancer (SCLC). " 01/01/2022 - "Small cell lung cancer (SCLC) displays marked arginine auxotrophy due to inactivation of the rate-limiting enzyme argininosuccinate synthetase 1 (ASS1), and as a consequence may be targeted with pegylated arginine deiminase or ADI-PEG20 (pegargiminase) and human recombinant pegylated arginases (rhArgPEG, BCT-100 and pegzilarginase). "

Related Drugs and Biologics

1.	Arginine (L-Arginine)
2.	ADI PEG20
3.	Enzymes
4.	Urea (Carbamide)
5.	Lyases
6.	Fluorouracil (Carac)
7.	Argininosuccinate Lyase
8.	Cisplatin (Platino)
9.	Pemetrexed (MTA)
10.	Citrulline

Related Therapies and Procedures

1.	Therapeutics
2.	Injections
3.	Drug Therapy (Chemotherapy)
4.	Oral Administration