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carbamoyl phosphate synthase-1 (NH3, CPS1)
Function:
- role in urea cycle of ureotelic animals where enzyme plays an important role in removing excess ammonia
- catalyzes formation of carbamoyl phosphate from CO2 & NH4+ in urea cycle
- requires N-acetylglutamate as an allosteric activator
2 ATP + NH3 + CO2 + H2O
2 ADP + phosphate + carbamoyl phosphate
Structure:
- type-1 glutamine amidotransferase domain is defective
- contains 2 ATP-grasp domains
- contains 1 glutamine amidotransferase type-1 domain
Compartment: mitochondria
Alternative splicing: named isoforms=2
Expression: primarily in liver & small intestine
Pathology:
- defects in CPS1 are the cause of CPS1 deficiency
- defects in CPS1 are associated with congenital alveolar capillary dysplasia
Interactions
molecular events
Related
carbamoylphosphate synthetase deficiency
urea cycle
General
carbamoyl phosphate synthase
mitochondrial protein
urea cycle enzyme
Properties
SIZE: entity length = 1500 aa
MW = 165 kD
COMPARTMENT: mitochondrial matrix
STATE: active state
MOTIF: Anthranilate phosphoribosyltransferase homolog {39-218}
Glutamine amidotransferase type-1 {219-404}
ATP-grasp domain
SITE: 551-743
Ser phosphorylation site {S898}
ATP-grasp domain
SITE: 1093-1284
allosteric site
FOR-BINDING-OF: N-acetylglutamate
Database Correlations
OMIM correlations
MORBIDMAP 608307
UniProt P31327
PFAM correlations
KEGG correlations
ENZYME 6.3.4.16
References
OMIM :accession 237300