카르바모일포스페이트:L-오르니틴카르바모일트랜스퍼라제

카르바모일포스페이트:L-오르니틴카르바모일트랜스퍼라제 화학적 특성, 용도, 생산

• 개요

  An enzyme that catalyzes the transfer of a carbamyl group from carbamyl phosphate to ornithine to form citrulline; it is a constituent reaction of the urea cycle. This enzyme normally occurs in the mitochondria of liver cells, but only to a minute extent in serum. Since it does not occur to a significant extent in serum, kidney, brain, etc., and only 1% or 2% of the hepatic concentration occurs in small intestine, its presence in serum is a specific indica_x0002_tion of damage to liver cells.

• Biochem/physiol Actions

  Mutations in ornithine transcarbamylase cause Ornithine transcarbamylase deficiency which prevents the clearance of the byproduct ammonia leading to hyperammonemia.

카르바모일포스페이트:L-오르니틴카르바모일트랜스퍼라제 공급 업체

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카르바모일포스페이트:L-오르니틴카르바모일트랜스퍼라제 관련 검색