ornithine transcarbamoylase

enzyme

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July 1, 2026

Quick Summary

Ornithine transcarbamoylase (OTC) is an enzyme crucial for the urea cycle in the liver. This cycle's primary function is to convert toxic ammonia, a byproduct of amino acid breakdown, into urea, which is then excreted in urine.12

A deficiency in OTC, known as OTC deficiency, is an X-linked genetic disorder that leads to a dangerous buildup of ammonia in the blood. This condition can cause severe symptoms, especially in males, and may require emergency treatment and long-term management through diet and medication. Liver transplantation can be a cure for some urea cycle disorders.2

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metabolism
Disposal of nitrogen
…in a reaction catalyzed by ornithine transcarbamoylase; the products are citrulline and inorganic phosphate [31]. Citrulline and aspartate formed from amino acids via step [26b] react to form argininosuccinate [32]; argininosuccinic acid synthetase catalyzes the reaction. Argininosuccinate splits into fumarate and arginine
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…in a reaction catalyzed by ornithine transcarbamoylase; the products are citrulline and inorganic phosphate [31]. Citrulline and aspartate formed from amino acids via step [26b] react to form argininosuccinate [32]; argininosuccinic acid synthetase catalyzes the reaction. Argininosuccinate splits into fumarate and arginine
Read More