What is 2-Keto-3-Methylvaleric Acid? High and low values | Lab results explained

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.

Higher values:

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.


– 2-Hydroxy-3-methylvalerate (YMDB01571) [L]

– (2R,3S)-2-Hydroxy-3-methylpentanoic acid [L]

– 2-Hydroxy-3-Methylvalerate/​Creatinine [Molar ratio] in Urine [L]


Test results may vary depending on your age, gender, health history, the method used for the test, and other things. Your test results may not mean you have a problem. Ask your healthcare provider what your test results mean for you.

The information on is NOT intended to replace a one-on-one relationship with a qualified health care professional and is not intended as medical advice.

Leave a Reply

Fill in your details below or click an icon to log in: Logo

You are commenting using your account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s

This site uses Akismet to reduce spam. Learn how your comment data is processed.

%d bloggers like this: