Protein Domain : IPR018223

Type:  Conserved_site Name:  Argininosuccinate synthase, 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
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0 Child Features

0 Contains

2 Cross Referencess

Identifier
PS00564
PS00565

2 Found Ins

DB identifier Type Name
IPR014729 Domain Rossmann-like alpha/beta/alpha sandwich fold
IPR001518 Family Argininosuccinate synthase

3 GO Annotations

GO Term Gene Name
GO:0004055 IPR018223
GO:0005524 IPR018223
GO:0006526 IPR018223

3 Ontology Annotations

GO Term Gene Name
GO:0004055 IPR018223
GO:0005524 IPR018223
GO:0006526 IPR018223

0 Parent Features

0 Proteins

2 Publications

First Author Title Year Journal Volume Pages PubMed ID
            2123815
            3133361