| Type: | Family | Name: | Argininosuccinate synthase, type 2 subfamily |
| Description: | Argininosuccinate synthase () (AS) is a urea cycle enzyme that catalyzes the penultimate step in arginine biosynthesis: the ATP-dependent ligation of citrulline to aspartate to form argininosuccinate, AMP and pyrophosphate [, ].In humans, a defect in the AS gene causes citrullinemia, a genetic disease characterised by severe vomiting spells and mental retardation.AS is a homotetrameric enzyme of chains of about 400 amino-acid residues. An arginine seems to be important for the enzyme's catalytic mechanism. The sequences of AS from various prokaryotes, archaebacteria and eukaryotes show significant similarity.This entry represents argininosuccinate synthase, type 2 subfamily. | Short Name: | Arg_succ_synth_type2_subfam |
