Product name3-Hydroxy-2-methylbutanoic acid, urinary metabolite
DescriptionUrinary metabolite excreted in abnormally high amounts in beta-ketothiolase deficiency
- 2-Methyl-3-hydroxybutyric Acid
- 3-Hydroxy-2-methylbutyric Acid
β-hydroxy carboxylic acid involved in isoleucine catabolism, as well as beta-oxidation of fatty acids and ketogenesis. Urinary metabolite excreted in abnormally high amounts in beta-ketothiolase deficiency.
Storage instructionsStore at -20°C. Store under desiccating conditions. The product can be stored for up to 12 months.
Wherever possible, you should prepare and use solutions on the same day. However, if you need to make up stock solutions in advance, we recommend that you store the solution as aliquots in tightly sealed vials at -20°C. Generally, these will be useable for up to one month. Before use, and prior to opening the vial we recommend that you allow your product to equilibrate to room temperature for at least 1 hour.
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This product has been referenced in:
- Heil M et al. Enantioselective analysis of ketone bodies in patients with beta-ketothiolase deficiency, medium-chain acyl coenzyme A dehydrogenase deficiency and ketonemic vomiting. J Chromatogr B Biomed Sci Appl 739:313-24 (2000). Read more (PubMed: 10755375) »