How Do You Spell ORNITHINE OXO ACID TRANSAMINASE?

1. Ornithine oxo acid transaminase, also known as ornithine aminotransferase (OAT), is an enzyme that plays a crucial role in the metabolism of amino acids and the urea cycle in organisms. It is specifically involved in the conversion of ornithine, a nonprotein amino acid, into glutamate, a key intermediate in amino acid metabolism.

   The function of ornithine oxo acid transaminase is to transfer the amino group from ornithine to α-ketoglutarate, forming glutamate and α-ketoacid as products. This transamination reaction is vital for the recycling of ornithine in the urea cycle, which allows the elimination of excess nitrogen as urea.

   OAT is primarily found in the mitochondria of liver cells, where it participates in the urea cycle and assists in the detoxification of nitrogenous compounds. Mutations in the gene encoding ornithine oxo acid transaminase can result in a rare genetic disorder called hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, which is characterized by elevated levels of ornithine, ammonia, and homocitrulline in the blood.

   In clinical settings, the activity of OAT can be measured by laboratory tests. Abnormalities in OAT levels may be indicative of certain liver diseases or urea cycle disorders. Understanding the role of ornithine oxo acid transaminase is crucial for studying the metabolism of amino acids and elucidating the pathophysiology of related disorders.

Common Misspellings for ORNITHINE OXO ACID TRANSAMINASE