Organic Acids, Plasma

2-Keto-3-Methylvaleric Acid

Optimal Result: 10 - 30 umol/L.

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.

References:

- Reference 1

- Reference 2

- Reference 3

What does it mean if your 2-Keto-3-Methylvaleric Acid result is too high?

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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