Conserved Site

Argininosuccinate synthase, conserved site (IPR018223)

Short name: Arginosuc_synth_CS


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.

GO terms

Biological Process

GO:0006526 arginine biosynthetic process

Molecular Function

GO:0005524 ATP binding
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.
PROSITE patterns
PROSITE patterns