Ornithine

transcarbamylase Ornithine transcarbamylase (OTC) is an enzyme that catalyzes the conversion of carbon skeleton from one molecule to another. This enzyme is important for the proper function of the urea cycle, a cellular process that helps organisms to break down nitrogen-containing compounds such as ammonia. OTC deficiency is the most common urea cycle disorder in humans and leads to a potentially life-threatening accumulation of ammonia in the blood. Diagnosis and management of OTC deficiency involves enzyme replacement therapies, dietary interventions, and medications. Because of the importance of OTC for proper functioning of the urea cycle, this enzyme can be used in research as a biomarker to study the health of the urea cycle in organisms and to identify potential new treatments for OTC deficiency.