ornithine transcarbamylase

GPTKB entity

Statements (23)
Predicate Object
gptkbp:instanceOf gptkb:enzyme
gptkbp:alternativeName gptkb:OTC
gptkbp:catalyzes conversion of ornithine and carbamoyl phosphate to citrulline
gptkbp:deficiencyCauses ornithine transcarbamylase deficiency
gptkbp:discoveredBy 1950s
gptkbp:EC_number 2.1.3.3
gptkbp:encodedBy gptkb:OTC_gene
gptkbp:foundIn mitochondria
https://www.w3.org/2000/01/rdf-schema#label ornithine transcarbamylase
gptkbp:involves gptkb:urea_cycle
gptkbp:locatedOnChromosome gptkb:X_chromosome_(Xp21.1)
gptkbp:molecularWeight approximately 36 kDa (monomer)
gptkbp:presentIn gptkb:small_intestine
liver
gptkbp:product phosphate
citrulline
gptkbp:requires ammonia detoxification
gptkbp:significance mutations cause hyperammonemia
gptkbp:structure homotrimer
gptkbp:substrate ornithine
carbamoyl phosphate
gptkbp:bfsParent gptkb:urea_cycle
gptkbp:bfsLayer 5