Domain Annotation: SCOP/SCOPe Classification SCOP-e Database Homepage

ChainsDomain InfoClassFoldSuperfamilyFamilyDomainSpeciesProvenance Source (Version)
Bd1j21b1 Alpha and beta proteins (a/b) Adenine nucleotide alpha hydrolase-like Adenine nucleotide alpha hydrolases-like N-type ATP pyrophosphatases Argininosuccinate synthetase, N-terminal domain (Thermus thermophilus ) [TaxId: 274 ], SCOPe (2.08)
Bd1j21b2 Alpha and beta proteins (a+b) Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain (Thermus thermophilus ) [TaxId: 274 ], SCOPe (2.08)
Cd1j21c1 Alpha and beta proteins (a/b) Adenine nucleotide alpha hydrolase-like Adenine nucleotide alpha hydrolases-like N-type ATP pyrophosphatases Argininosuccinate synthetase, N-terminal domain (Thermus thermophilus ) [TaxId: 274 ], SCOPe (2.08)
Cd1j21c2 Alpha and beta proteins (a+b) Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain (Thermus thermophilus ) [TaxId: 274 ], SCOPe (2.08)
Dd1j21d1 Alpha and beta proteins (a/b) Adenine nucleotide alpha hydrolase-like Adenine nucleotide alpha hydrolases-like N-type ATP pyrophosphatases Argininosuccinate synthetase, N-terminal domain (Thermus thermophilus ) [TaxId: 274 ], SCOPe (2.08)
Dd1j21d2 Alpha and beta proteins (a+b) Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain (Thermus thermophilus ) [TaxId: 274 ], SCOPe (2.08)
Ad1j21a1 Alpha and beta proteins (a/b) Adenine nucleotide alpha hydrolase-like Adenine nucleotide alpha hydrolases-like N-type ATP pyrophosphatases Argininosuccinate synthetase, N-terminal domain (Thermus thermophilus ) [TaxId: 274 ], SCOPe (2.08)
Ad1j21a2 Alpha and beta proteins (a+b) Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain Argininosuccinate synthetase, C-terminal domain (Thermus thermophilus ) [TaxId: 274 ], SCOPe (2.08)

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

ChainsTypeFamily Name Domain Identifier Family IdentifierProvenance Source (Version)
BSCOP2B SuperfamilyArgininosuccinate synthetase, C-terminal domain 8032266 3001063 SCOP2B (2022-06-29)
BSCOP2B SuperfamilyAdenine nucleotide alpha hydrolases-like 8032265 3001593 SCOP2B (2022-06-29)
CSCOP2B SuperfamilyArgininosuccinate synthetase, C-terminal domain 8032266 3001063 SCOP2B (2022-06-29)
CSCOP2B SuperfamilyAdenine nucleotide alpha hydrolases-like 8032265 3001593 SCOP2B (2022-06-29)
DSCOP2B SuperfamilyArgininosuccinate synthetase, C-terminal domain 8032266 3001063 SCOP2B (2022-06-29)
DSCOP2B SuperfamilyAdenine nucleotide alpha hydrolases-like 8032265 3001593 SCOP2B (2022-06-29)
ASCOP2B SuperfamilyAdenine nucleotide alpha hydrolases-like 8032265 3001593 SCOP2B (2022-06-29)
ASCOP2B SuperfamilyArgininosuccinate synthetase, C-terminal domain 8032266 3001063 SCOP2B (2022-06-29)

Domain Annotation: ECOD Classification ECOD Database Homepage

ChainsFamily NameDomain Identifier ArchitecturePossible HomologyHomologyTopologyFamilyProvenance Source (Version)
BArginosuc_synth_Ce1j21B1 A: a+b complex topologyX: Argininosuccinate synthetase-C (From Topology)H: Argininosuccinate synthetase-C (From Topology)T: Argininosuccinate synthetase-CF: Arginosuc_synth_CECOD (1.6)
BArginosuc_synth_Ne1j21B2 A: a/b three-layered sandwichesX: HUP domain-likeH: HUP domains (From Topology)T: HUP domainsF: Arginosuc_synth_NECOD (1.6)
CArginosuc_synth_Ce1j21C1 A: a+b complex topologyX: Argininosuccinate synthetase-C (From Topology)H: Argininosuccinate synthetase-C (From Topology)T: Argininosuccinate synthetase-CF: Arginosuc_synth_CECOD (1.6)
CArginosuc_synth_Ne1j21C2 A: a/b three-layered sandwichesX: HUP domain-likeH: HUP domains (From Topology)T: HUP domainsF: Arginosuc_synth_NECOD (1.6)
DArginosuc_synth_Ce1j21D1 A: a+b complex topologyX: Argininosuccinate synthetase-C (From Topology)H: Argininosuccinate synthetase-C (From Topology)T: Argininosuccinate synthetase-CF: Arginosuc_synth_CECOD (1.6)
DArginosuc_synth_Ne1j21D2 A: a/b three-layered sandwichesX: HUP domain-likeH: HUP domains (From Topology)T: HUP domainsF: Arginosuc_synth_NECOD (1.6)
AArginosuc_synth_Ce1j21A1 A: a+b complex topologyX: Argininosuccinate synthetase-C (From Topology)H: Argininosuccinate synthetase-C (From Topology)T: Argininosuccinate synthetase-CF: Arginosuc_synth_CECOD (1.6)
AArginosuc_synth_Ne1j21A2 A: a/b three-layered sandwichesX: HUP domain-likeH: HUP domains (From Topology)T: HUP domainsF: Arginosuc_synth_NECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Protein Family Annotation Pfam Database Homepage

ChainsAccessionNameDescriptionCommentsSource
A, B, C, D
PF20979Arginosuccinate synthase C-terminal domain (Arginosuc_syn_C)Arginosuccinate synthase C-terminal domainThis entry represents the C-terminal domain of the Arginosuccinate synthase enzyme.Domain
A, B, C, D
PF00764Arginosuccinate synthase N-terminal HUP domain (Arginosuc_synth)Arginosuccinate synthase N-terminal HUP domainThis entry represents the N-terminal domain of the Arginosuccinate synthase enzyme. This domain is part of the HUP superfamily.Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

ChainsPolymerMolecular FunctionBiological ProcessCellular Component
A, B, C, D
Argininosuccinate Synthetase

Structure Motif Annotation: Mechanism and Catalytic Site Atlas M-CSA Database Homepage

ChainsEnzyme NameDescriptionCatalytic Residues
argininosuccinate synthetase  M-CSA #316

Argininosuccinate synthase (EC:6.3.4.5) (AS) is a urea cycle enzyme that catalyzes the penultimate (seventh) step in arginine biosynthesis: the condensation of glutamate and citrulline to form agininosuccinate, AMP and diphosphate.

In humans, a defect in the AS gene causes citrullinemia, a genetic disease characterised by severe vomiting spells and mental retardation [PMID:19006241].

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.

Although there are two sub-types of argininosuccinate synthase, they appear to have the same mechanism and so are both represented by this entry.

Defined by 4 residues: ASP:A-12ARG:A-92ASP:A-121SER:A-173
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