CATALYTIC ACTIVITY: ATP + L-citrulline + L-aspartate = AMP + diphosphate + L-argininosuccinate. * PATHWAY: UREA CYCLE, PENULTIMATE STEP OF THE ARGININE BIOSYNTHETIC PATHWAY. * SUBUNIT: Homotetramer. * DISEASE: Defects in ASS are the cause of citrullinemia, an autosomal recessive disease characterized by severe vomiting spells and mental retardation. * SIMILARITY: BELONGS TO THE ARGININOSUCCINATE SYNTHASE FAMILY. SUBFAMILY 1. |
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25-JAN-03
Last Update 13-Mar-03