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

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

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.

See also

• Keto acid
• Leucine metabolism
• Maple syrup urine disease

References

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External links

• PubChem Compound Summary for CID 58, α-Ketoisovaleric acid
• KEGG Compound: C00141 α-Ketoisovaleric acid

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  Alpha-Ketovaline