Argininosuccinic aciduria

Argininosuccinic aciduria (ASA) is an inherited disease characterized by vomiting, liver disease, and intellectual disabilities. It involves defects in an enzyme called argininosuccinate lyase (ASL). This enzyme is important for breaking down nitrogen (found in proteins) so that it does not build up as ammonia. The symptoms of argininosuccinic aciduria are due to the toxic accumulation of ammonia, particularly in the liver and nervous system. Argininosuccinic aciduria is also known as argininosuccinate lyase (ASL) deficiency. View testing options