Protein Domain : Argininosuccinate synthase, conserved site IPR018223

Type  Conserved_site
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.
Short Name  Arginosuc_synth_CS

0 Child Features

0 Gene Families

201 Genes

3 Ontology Annotations

0 Parent Features

13 Publications

USDA
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