Anti-Ornithine transcarbamylase / OTCase / OTC

In 1X PBS, pH 7.4, with 0.09% sodium azide. Ornithine transcarbamylase is an enzyme that is responsible for catalyzing the reaction between ornithine and carbamoyl phosphate, ultimately forming citrulline. Citrulline then goes on to participate in the urea cycle, where it helps convert toxic ammonia into urea, which can be safely excreted by the body. A deficiency in this enzyme can lead to a rare genetic disorder known as ornithine transcarbamylase deficiency. Individuals with this condition are unable to properly metabolize ammonia, leading to a buildup of toxic levels in the blood. This can result in a range of symptoms, from mild to severe, including fatigue, seizures, and even coma if left untreated. Protein function: Catalyzes the second step of the urea cycle, the condensation of carbamoyl phosphate with L-ornithine to form L-citrulline (PubMed:2556444, PubMed:6372096, PubMed:8112735). The urea cycle ensures the detoxification of ammonia by converting it to urea for excretion (PubMed:2556444). [The UniProt Consortium]