2-Hydroxyisovaleric acid (aka 2-Hydroxy-3-methylbutyric acid) is a branched-chain amino acid metabolite.
2-hydroxy-3-methylbutyric acid is a valine derivative that is valine in which the amino group has been replaced by a ‘hydroxy’ group.
Moderate increase may result from:
- lactic acidosis
- episodic ketosis
- thiamine/lipoic acid deficiency
Patients with slight to moderate elevations may use dietary supplements containing thiamine to improve clinical symptoms.
Significant elevations are associated with:
- A significant increase of branched-chain amino acid metabolites is associated with the genetic disorders, maple syrup urine disease (MSUD) and pyruvate dehydrogenase deficiency.
- Elevated levels have been linked to short bowel syndrome.
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2-Hydroxybutyric, 2-Hydroxyhippuric, 2-Hydroxyisocaproic, 2-Hydroxyisovaleric, 2-Hydroxyphenylacetic, 2-Oxo-4-methiolbutyric, 2-Oxoglutaric, 2-Oxoisocaproic, 2-Oxoisovaleric, 3-Hydroxy-3-methylglutaric (HMG), 3-Hydroxybutyric, 3-Hydroxyglutaric, 3-Indoleacetic, 3-Methyl-2-oxovaleric, 3-Methylglutaconic, 3-Methylglutaric, 3-Oxoglutaric, 4-Cresol, 4-Hydroxybenzoic, 4-Hydroxybutyric, 4-Hydroxyhippuric, 4-Hydroxyphenylacetic, 4-Hydroxyphenyllactic, 5-Hydroxyindoleacetic (5-HIAA), 5-Hydroxymethyl-2-furoic, Acetoacetic, Aconitic, Adipic, Arabinose, Ascorbic, Carboxycitric, Citramalic, Citric, DHPPA (Beneficial Bacteria), Ethylmalonic, Fumaric, Furan-2,5-dicarboxylic, Furancarbonylglycine, Glutaric, Glyceric, Glycolic, Hippuric, Homogentisic, Homovanillic (HVA), HPHPA, HVA / VMA Ratio, HVA/ DOPAC Ratio, Kynurenic, Lactic, Malic, Malonic, Mandelic, Methylcitric, Methylmalonic, Methylsuccinic, N-Acetylaspartic, N-Acetylcysteine (NAC), Orotic, Oxalic, Pantothenic (B5), Phenyllactic, Phenylpyruvic, Phosphoric, Pyridoxic (B6), Pyroglutamic, Pyruvic, Quinolinic, Quinolinic / 5-HIAA Ratio, Sebacic, Suberic, Succinic, Tartaric, Thymine, Tricarballylic, Uracil, Vanillylmandelic (VMA)
