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.
References:
- 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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1-Methylhistidine, 1-Methylhistidine (Plasma), 2-Methylhippuric Acid, 2-Methylsuccinic Acid, 3,4-Dihydroxyhydrocinnamic Acid, 3,5-Dihydroxybenzoic Acid, 3-Methylhistidine (Plasma), 3-Phenylpropionylglycine, 4-Hydroxybenzoic Acid, 4-Hydroxyphenylacetic Acid, 4-Hydroxyphenylpyruvic Acid, 5-Hydroxyindoleacetic Acid, 8-Hydroxy-2'-deoxyguanosine, a-Hydroxybutyric Acid, a-Ketobutyric Acid, a-Ketoglutaric Acid, a-Ketoisovaleric Acid, a-Aminoadipic Acid (Plasma), a-Keto-b-methylvaleric Acid, a-Ketoisocaproic Acid, Adipic Acid, Alanine, Alanine (Plasma), Aldosterone, Anserine (Plasma), Anthranilic Acid, Arabinitol, Arginine (Plasma), Arginosuccinic Acid, Arginosuccinic Acid (Plasma), Asparagine (Plasma), Aspartic Acid (Plasma), b-Hydroxybutyric Acid, b-Alanine (Plasma), b-Hydroxyisovaleric Acid, Benzoic Acid, Benzoylform, Branched Chain Alpha-Keto Organic Acids, Carnosine, Carnosine (Plasma), cis-Aconitic Acid, Citric Acid, Citrulline (Plasma), Cortisol, Cortisone, Creatinine, Cystathionine (Plasma), Cystine (Plasma), D-Lactic Acid, Equol, Ethanolamine (Plasma), Ethylmalonic Acid, Formiminoglutamic Acid, Fructose, Fumaric Acid, g-Aminobutyric Acid (Plasma), Glucaric Acid, Glucose, Glutamic Acid (Plasma), Glutamine (Plasma), Glutamine / Glutamate Ratio (Plasma), Glutaric Acid, Glycine (Plasma), Glycylproline (Plasma), Hexanoylglycine, Hippuric Acid, Histidine (Plasma), Homocitrulline (Plasma), Homocystine (Plasma), Homogentisic Acid, Homovanillic Acid, Hydroxykynurenine, Hydroxyproline, Hydroxyproline (Plasma), Indoleacetic Acid, Isocitric Acid, Isoleucine/allo-Isoleucine (Plasma), KT Ratio (Plasma), Kynurenic Acid, Kynurenine (Plasma), Lactic Acid, Leucine (Plasma), Lysine (Plasma), Malic Acid, Mandelic Acid, Methionine (Plasma), Methylmalonic Acid, Microalbumin, Ornithine, Ornithine (Plasma), Orotic Acid, Oxalic Acid, pH, Phenylacetic Acid, Phenylalanine (Plasma), Phosphate, Phosphoethanolamine (Plasma), Picolinic Acid, Pimelic Acid, Proline (Plasma), Pyridoxic Acid, Pyroglutamic Acid, Pyruvic Acid, Quercetin, Quinolinic Acid, Sarcosine (Plasma), Sebacic Acid, Serine (Plasma), Suberic Acid, Suberylglycine, Succinic Acid, Sulfocysteine (Plasma), Tartaric Acid, Taurine (Plasma), Threonine (Plasma), Total Branched Chain Amino Acids (Plasma), Tryptophan (Plasma), Tyrosine (Plasma), Valine (Plasma), Vannilylmandelic Acid, Xanthurenic Acid
