ASL

ASL (Argininosuccinate Lyase) is a protein coding gene that encodes a member of the lyase 1 family. The encoded protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. Mutations in this gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency. Diseases associated with ASL include Argininosuccinic Aciduria and Urea Cycle Disorder. Among its related pathways are Arginine biosynthesis and Viral mRNA Translation.