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.
Understand and improve your laboratory results with our health dashboard.
Upload your lab reports and get your interpretation today.
Our technology helps to understand, combine, track, organize, and act on your medical lab test results.
- 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.
Interpret Your Lab Results
Upload your lab report, and we'll interpret and provide you with recommendations today.
Get StartedOur specialized data entry service is designed to seamlessly integrate your laboratory results into your private dashboard. Just send in your lab test results—whether it's an image or a file—and our skilled data entry team will handle the rest. We accommodate various file formats like PDFs, JPGs, and Excel.
The first report is complimentary. After that, the data entry service is priced at $15 per report, unless it's part of your subscribed plan. It's an ideal solution whether you have numerous reports to upload or if your schedule doesn't permit self-data entry.
We strive to make the data entry process easy for you. Whether by offering dozens of templates to choose from that pre-populate the most popular laboratory panels or by giving you instant feedback on the entered values. Our data entry forms are an easy, fast, and convenient way to enter the reports yourself. There is no limit on how many lab reports you can upload.
Personal plans
track personal results
Professional Plan
track multiple client's results
$15/month
$250/once
own it for life
$45/month
for health professionals
Personal Account
$15/month
for personal lab results
$250/once
own it for life
Level up your lab report analysis with our Pro plan, built for health practitioners like you.
Health Business Account
$45/month
Unlock additional Pro plans when you sign up.
At HealthMatters, we're committed to maintaining the security and confidentiality of your personal information. We've put industry-leading security standards in place to help protect against the loss, misuse, or alteration of the information under our control. We use procedural, physical, and electronic security methods designed to prevent unauthorized people from getting access to this information. Our internal code of conduct adds additional privacy protection. All data is backed up multiple times a day and encrypted using SSL certificates. See our Privacy Policy for more details.
1-Methylhistidine, 1-Methylhistidine (Plasma), 2-,3-, and 4-Methylhippuric acid, 2-Methylhippuric Acid, 2-Methylsuccinic Acid, 3,4-Dihydroxyhydrocinnamic Acid, 3,5-Dihydroxybenzoic Acid, 3-Methylhistidine, 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-Keto-b-methylvaleric Acid, a-Ketobutyric Acid, a-Ketoglutaric Acid, a-Ketoisocaproic Acid, a-Ketoisovaleric Acid, a-Aminoadipic Acid, a-Aminoadipic Acid (Plasma), Adipic Acid, Alanine, Alanine (Plasma), Aldosterone, Anserine (Plasma), Anthranilic Acid, Arabinitol, Arginine, Arginine (Plasma), Arginosuccinic Acid, Arginosuccinic Acid (Plasma), Asparagine, Asparagine (Plasma), Aspartic Acid (Plasma), b-Hydroxybutyric Acid, b-Hydroxyisovaleric Acid, b-Alanine, b-Alanine (Plasma), Benzoic Acid, Benzoylform, Branched Chain Alpha-Keto Organic Acids, Carnosine, Carnosine (Plasma), cis-Aconitic Acid, Citric Acid, Citrulline, Citrulline (Plasma), Cortisol, Cortisone, Creatinine, Cystathionine, Cystathionine (Plasma), Cystine, Cystine (Plasma), D-Lactic Acid, Equol, Ethanolamine, Ethanolamine (Plasma), Ethylmalonic Acid, Formiminoglutamic Acid, Fructose, Fumaric Acid, g-Aminobutyric Acid, g-Aminobutyric Acid (Plasma), Glucaric Acid, Glucose, Glutamic Acid, Glutamic Acid (Plasma), Glutamine, Glutamine (Plasma), Glutamine / Glutamate Ratio, Glutamine / Glutamate Ratio (Plasma), Glutaric Acid, Glycine, Glycine (Plasma), Glycylproline, Glycylproline (Plasma), Hexanoylglycine, Hippuric Acid, Histidine, Histidine (Plasma), Homocitrulline (Plasma), Homocystine, Homocystine (Plasma), Homogentisic Acid, Homovanillic Acid, Hydroxykynurenine, Hydroxyproline, Hydroxyproline (Plasma), Indoleacetic Acid, Isocitric Acid, Isoleucine/allo-Isoleucine, Isoleucine/allo-Isoleucine (Plasma), KT Ratio, KT Ratio (Plasma), Kynurenic Acid, Kynurenine, Kynurenine (Plasma), Lactic Acid, Leucine, Leucine (Plasma), Lysine, Lysine (Plasma), Malic Acid, Mandelic Acid, Methionine, Methionine (Plasma), Methylmalonic Acid, Microalbumin, Ornithine, Ornithine (Ornithine transcarbamylase), Ornithine (Plasma), Orotic Acid, Oxalic Acid, pH, Phenylacetic Acid, Phenylalanine, Phenylalanine (Plasma), Phosphate, Phosphoethanolamine, Phosphoethanolamine (Plasma), Picolinic Acid, Pimelic Acid, Proline, Proline (Plasma), Pyridoxic Acid, Pyroglutamic Acid, Pyruvic Acid, Quercetin, Quinolinic Acid, Sarcosine, Sarcosine (Plasma), Sebacic Acid, Serine, Serine (Plasma), Suberic Acid, Suberylglycine, Succinic Acid, Sulfocysteine, Sulfocysteine (Plasma), Tartaric Acid, Taurine, Taurine (Plasma), Threonine, Threonine (Plasma), Total Branched Chain Amino Acids, Total Branched Chain Amino Acids (Plasma), Tryptophan, Tryptophan (Plasma), Tyrosine, Tyrosine (Plasma), Valine, Valine (Plasma), Vannilylmandelic Acid, Xanthurenic Acid