Accession NumberHMDB00408
Common_Name2-Methyl-3-ketovaleric acid
Description2-Methyl-3-ketovaleric acid is a metabolite of beta-leucine in the beta-keto pathway of leucine metabolism. 2-Methyl-3-ketovaleric acid is a known pathological metabolite and associated with propionic acidemia, especially during periods of ketoacidosis; a severe deficiency of propionyl-CoA carboxylase in cultured fibroblasts has been detected in some neonatal cases. (PMID 7389125, 6820422, 7119896, 7430116, 3356699) 2-Methyl-3-ketovaleric acid induces a strong activity of inosine-5'-monophosphate dehydrogenase (IMPDH; the rate-limiting enzyme in GTP synthesis), mimicking glucose. (PMID: 11145582)
Chemical_IUPAC_Name2-methyl-3-oxo-pentanoic acid
Chemical FormulaC6H10O3
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Predicted 1H NMR SpectrumDownload
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Predicted 13C NMR PeaklistDownload
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