Otc
Function
Catalyzes the second step of the urea cycle, the condensation of carbamoyl phosphate with L-ornithine to form L-citrulline. The urea cycle ensures the detoxification of ammonia by converting it to urea for excretion.
Involvement in disease
Defects in Otc are the cause of the Sparse fur (spf) phenotype. Spf mouse have an OTCase with an overall decrease in activity, and altered substrate affinity.
Pathway
Nitrogen metabolism; urea cycle; L-citrulline from L-ornithine and carbamoyl phosphate: step 1/1.
Post-translational modifications
Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.
Sequence Similarities
Belongs to the aspartate/ornithine carbamoyltransferase superfamily. OTCase family.
Cellular localization
- Mitochondrion matrix
Alternative names
OTCase, Otc