Anti-Argininosuccinate Lyase antibody

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Argininosuccinate Lyase often referred to as ASL or 'lyase' is an enzyme that plays a mechanical role in the urea cycle catalyzing the conversion of argininosuccinate into arginine and fumarate. It has a molecular mass around 51 kDa. ASL is mainly expressed in the liver but is also present in the kidney and other tissues. The proper functioning of ASL is essential for maintaining the urea cycle which detoxifies ammonia in the body.

Biological function summary

This enzyme is important for the breakdown of nitrogen molecules impacting amino acid metabolism. ASL does not operate as part of a larger complex but acts independently to facilitate the removal of nitrogenous waste. Through its action it produces arginine which serves as a vital precursor for the production of nitric oxide a critical signaling molecule in many physiological processes.

Pathways

ASL is integral to the urea cycle and amino acid metabolic pathways. The urea cycle is fundamental for ammonia detoxification while amino acid metabolic pathways involve protein turnover and energy production. Within the urea cycle ASL works alongside enzymes like argininosuccinate synthetase and arginase playing a pivotal role in converting excess nitrogen to urea which the body then excretes.

Associated diseases and disorders

Defects in ASL can lead to argininosuccinate lyase deficiency a rare disorder that causes hyperammonemia due to urea cycle dysfunction. This deficiency associates with elevated levels of argininosuccinate in the blood and urine. Additionally ASL has been implicated in vascular diseases due to its role in arginine and nitric oxide production. Its connection to nitric oxide emphasizes the potential impact on cardiovascular health with relations to proteins such as nitric oxide synthase.