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