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Argininosuccinate synthase (EC 6.3.4.5) (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 [1,2].

In humans, a defect in the AS gene causes citrullinemia, a genetic disease characterized 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. We selected two signature patterns for AS. The first is a highly conserved stretch of nine residues located in the N-terminal extremity of these enzymes, the second is derived from a conserved region which contains one of the conserved arginine residues.

December 2004 / Patterns and text revised.

PROSITE methods (with tools and information) covered by this documentation:

ARGININOSUCCIN_SYN_1, PS00564; Argininosuccinate synthase signature 1  (PATTERN)

Consensus pattern: [ASL]-[FY]-S-G-G-[LV]-D-T-[ST] Sequences known to belong to this class detected by the pattern: ALL Other sequence(s) detected in Swiss-Prot: NONE.

• Retrieve an alignment of Swiss-Prot true positive hits:   Clustal format, color, condensed view  / Clustal format, color  / Clustal format, plain text  / Fasta format • Retrieve the sequence logo from the alignment • Taxonomic tree view of all Swiss-Prot/TrEMBL entries matching PS00564 • Retrieve a list of all Swiss-Prot/TrEMBL entries matching PS00564 • Scan Swiss-Prot/TrEMBL entries against PS00564 • view ligand binding statistics

Matching PDB structures: 1J1Z 1J20 1J21 1K92 ... [ALL]

ARGININOSUCCIN_SYN_2, PS00565; Argininosuccinate synthase signature 2  (PATTERN)

Consensus pattern: G-x-T-x-[KRM]-G-N-D-x(2)-R-F Sequences known to belong to this class detected by the pattern: ALL Other sequence(s) detected in Swiss-Prot: NONE.

• Retrieve an alignment of Swiss-Prot true positive hits:   Clustal format, color, condensed view  / Clustal format, color  / Clustal format, plain text  / Fasta format • Retrieve the sequence logo from the alignment • Taxonomic tree view of all Swiss-Prot/TrEMBL entries matching PS00565 • Retrieve a list of all Swiss-Prot/TrEMBL entries matching PS00565 • Scan Swiss-Prot/TrEMBL entries against PS00565 • view ligand binding statistics

Matching PDB structures: 1J1Z 1J20 1J21 1K92 ... [ALL]

1	Authors	van Vliet F., Crabeel M., Boyen A., Tricot C., Stalon V., Falmagne P., Nakamura Y., Baumberg S., Glansdorff N.
	Source	Gene 95:99-104(1990).

2	Authors	Morris C.J., Reeve J.N.
	Title	Conservation of structure in the human gene encoding argininosuccinate synthetase and the argG genes of the archaebacteria Methanosarcina barkeri MS and Methanococcus vannielii.
	Source	J. Bacteriol. 170:3125-3130(1988).
	PubMed ID	3133361

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