ornithine carbamoyltransferase (OTCase, ornithine transcarbamylase, OTC)

Function

• nitrogen metabolism; urea cycle
• catalyzes condensation of ornithine with carbamoyl phosphate to form citrulline in urea cycle

carbamoyl phosphate + L-ornithine <--> phosphate + L-citrulline

Structure

• homotrimer
• belongs to the ATCase/OTCase family

Compartment

mitochondrial matrix

Expression

mainly in liver & intestinal mucosa

Pathology

• defects in OTC are the cause of ornithine hyperammonemia

More general terms

• transferase
• mitochondrial protein
• urea cycle enzyme

Additional terms

• urea cycle

Database

• UniProt: http://www.uniprot.org/uniprot/P00480.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:5009
• OMIM: https://mirror.omim.org/entry/300461
• OMIM: https://mirror.omim.org/entry/311250