Other names: HMVA / 2-Hydroxy-3-methylpentanoic acid
2-Hydroxy-3-methylpentanoic acid or 2-hydroxy-3-methylvaleric acid (HMVA) is an organic acid generated by L-isoleucine metabolism. It is derived from the reduction of 2-Keto-3-methylvaleric acid (KMVA), possibly through the action of a lactate dehydrogenase.
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2-hydroxy-3-methylvaleric acid (HMVA) is an acid generated by L-isoleucine oxidation pathway and involves four stereoisomers (2S,3S-HMVA, 2R,3R-HMVA, 2S,3R-HMVA and 2R,3S-HMVA). Increased levels of HMVA are found in people with maple syrup urine disease (MSUD), an inherited metabolic disease characterized by sweet-smelling urine, ketoacidosis, seizures, and neurological dysfunction.
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