Carbamoyl phosphate synthetase I

URI: https://gptkb.org/entity/Carbamoyl_phosphate_synthetase_I
GPTKB entity
Statements (33)
Predicate Object
gptkbp:instanceOf gptkb:enzyme
gptkbp:alternativeName gptkb:CPS1
CPS I
gptkbp:catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate
gptkbp:cofactor Mg2+
gptkbp:compatibleWith cytosol
gptkbp:deficiencyCauses hyperammonemia
gptkbp:EC_number 6.3.4.16
gptkbp:enzymeClassification gptkb:ligase
gptkbp:foundIn liver
gptkbp:gene gptkb:CPS1
https://www.w3.org/2000/01/rdf-schema#label Carbamoyl phosphate synthetase I
gptkbp:locatedOnChromosome 2q34
gptkbp:location mitochondria
gptkbp:molecularWeight approximately 160 kDa
gptkbp:not_involved_in pyrimidine synthesis
gptkbp:OMIM 237300
gptkbp:participatedIn gptkb:urea_cycle
gptkbp:product phosphate
carbamoyl phosphate
2 ADP
gptkbp:regulates activated by N-acetylglutamate
gptkbp:requires N-acetylglutamate as an allosteric activator
gptkbp:species gptkb:Homo_sapiens
gptkbp:structure multimeric protein
gptkbp:substrate ammonia
bicarbonate
2 ATP
gptkbp:suffersFrom gptkb:Carbamoyl_phosphate_synthetase_I_deficiency
gptkbp:UniProtID gptkb:P31327
gptkbp:bfsParent gptkb:CPS1
gptkb:CPS-1
gptkbp:bfsLayer 6