ASL : argininosuccinate lyase
Description
The ASL (argininosuccinate lyase) is a protein-coding gene located on chromosome 7.
The ASL gene provides instructions for making the enzyme argininosuccinate lyase. This enzyme is a key player in the urea cycle, a series of reactions taking place in liver cells. The urea cycle handles excess nitrogen generated when the body utilizes protein, transforming it into urea, which is then eliminated by the kidneys. This process prevents the buildup of harmful ammonia. The primary role of argininosuccinate lyase is to initiate the reaction producing the amino acid arginine from argininosuccinate, a molecule carrying waste nitrogen collected during the urea cycle. Arginine is subsequently broken down into urea for excretion and ornithine, which restarts the urea cycle. Beyond the liver, argininosuccinate lyase facilitates the movement of arginine into cells throughout the body, where it is used to make nitric oxide. Nitric oxide plays a crucial role in regulating blood flow and blood pressure.
The ASL gene encodes argininosuccinate lyase (ASL), an enzyme that catalyzes the reversible cleavage of L-argininosuccinate to fumarate and L-arginine. This reaction is an intermediate step in the urea cycle, which is primarily involved in hepatic nitrogen detoxification into excretable urea. ASL also plays a role in de novo L-arginine synthesis in nonhepatic tissues. ASL acts as a crucial regulator of intracellular and extracellular L-arginine pools. In the citrulline-nitric oxide cycle, ASL forms tissue-specific multiprotein complexes with argininosuccinate synthase (ASS1), transport protein SLC7A1, and nitric oxide synthase (NOS1, NOS2, or NOS3). These complexes enable cell-autonomous L-arginine synthesis and channel extracellular L-arginine to the nitric oxide synthesis pathway.
ASL is also known as ASAL.
