Argininosuccinate synthase, type 1 subfamily (IPR023434)

Short name: Arginosuc_synth_type_1_subfam

Overlapping homologous superfamilies

Family relationships


Argininosuccinate synthase (EC: (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 [PMID: 2123815, PMID: 3133361].

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 1 subfamily.

GO terms

Biological Process

No terms assigned in this category.

Molecular Function

GO:0004055 argininosuccinate synthase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.
  • cd01999 (Argininosuccinate_Synthase)