Α-Ketoisovaleric acid

Α-Ketoisovaleric acid (also known as 3-methyl-2-oxobutanoic acid) is an intermediate in the metabolism of amino acids. It is a keto acid, a type of organic compound that contains both a carboxylic acid functional group and a ketone functional group.

Metabolism[edit | edit source]

Α-Ketoisovaleric acid is produced from the transamination of leucine, an essential amino acid. This process is catalyzed by the enzyme branched-chain amino acid transaminase. The resulting α-ketoisovaleric acid can then be decarboxylated to produce isovaleryl-CoA, which is further metabolized in the mitochondria through the process of beta oxidation.

Clinical significance[edit | edit source]

Abnormal levels of α-ketoisovaleric acid can be indicative of certain metabolic disorders. For example, elevated levels can be a sign of maple syrup urine disease, a rare inherited metabolic disorder characterized by a deficiency in the enzyme branched-chain alpha-keto acid dehydrogenase complex that is responsible for the breakdown of the branched-chain amino acids leucine, isoleucine, and valine.

References[edit | edit source]

External links[edit | edit source]

This biochemistry article is a stub. You can help WikiMD by expanding it.

This pharmacology-related article is a stub. You can help WikiMD by expanding it.

Navigation: Wellness - Encyclopedia - Health topics - Disease Index‏‎ - Drugs - World Directory - Gray's Anatomy - Keto diet - Recipes

Search WikiMD

Ad.Tired of being Overweight? Try W8MD's physician weight loss program.
Semaglutide (Ozempic / Wegovy and Tirzepatide (Mounjaro) available.
Advertise on WikiMD

WikiMD is not a substitute for professional medical advice. See full disclaimer.

Credits:Most images are courtesy of Wikimedia commons, and templates Wikipedia, licensed under CC BY SA or similar.