b-Hydroxyisovaleric Acid [aka 3-Hydroxyisovaleric Acid (3-HIA)] is formed from the metabolism of the branched-chain amino acid leucine. Methylcrotonyl-CoA carboxylase catalyzes an essential step in this pathway and is biotin dependent. Reduced activity of this enzyme leads to an alternate pathway of metabolism resulting in 3-hydroxyisovaleric acid.
- Increased urinary excretion of 3-hydroxyisovaleric acid is used as an indicator of biotin deficiency.
- Urinary excretion of 3-hydroxyisovaleric acid was greater in smokers.
- People with multiple acyl-CoA dehydrogenase deficiency (MADD) shows elevations of ethylmalonic acid, glutaric acid, 3-hydroxyisovaleric acid, and other key markers.
- Animal research found feeding a ketogenic-type diet, low in carbohydrates and high in fat, exaggerated biotin deficiencies. A ketogenic diet may increase biotin need.
- Mock NI, Malik MI, Stumbo PJ, Bishop WP, Mock DM. Increased urinary excretion of 3-hydroxyisovaleric acid and decreased urinary excretion of biotin are sensitive early indicators of decreased biotin status in experimental biotin deficiency. Am J Clin Nutr. 1997;65(4):951-958.
- Mock DM, Quirk JG, Mock NI. Marginal biotin deficiency during normal pregnancy. Am J Clin Nutr. 2002;75(2):295-299.
- Sealey WM, Teague AM, Stratton SL, Mock DM. Smoking accelerates biotin catabolism in women. Am J Clin Nutr. 2004;80(4):932-935.
- Mock DM, Dyken ME. Biotin catabolism is accelerated in adults receiving long-term therapy with anticonvulsants. Neurology. 1997;49(5):1444-1447.
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- The urinary excretion of 3-HIA has been shown to be an early and sensitive indicator for marginal biotin deficiency.
- Elevated levels of 3-HIA in pregnant women reflect reduced or marginal biotin status.
- Smoking and anticonvulsant medication can also increase this metabolite as a reflection of accelerated biotin metabolism and therefore marginal deficiency.
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