Accession NumberHMDB00754
Common_Name3-Hydroxyisovaleric acid
Description3-Hydroxyisovaleric acid is a normal human metabolite excreted in the urine. Elevated levels of this compound are found in several inherited disorders such as Dihydrolipoamide dehydrogenase Deficiency, 3-Methylcrotonyl-CoA carboxylase 1 deficiency, 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (3-hydroxy-3-methylglutaryl -CoA lyase Deficiency, Biotinidase deficiency multiple carboxylase deficiency late-onset , Late onset multiple carboxylase deficiency, HolMcarboxylase synthetase deficiency, 3-Methylcrotonyl-CoA carboxylase 2 deficiency. 3-Hydroxyisovaleric acid is also elevated in smokers, in subjects undergoing long-term anticonvulsant therapy with carbamazepine and/or phenytoin. These levels are elevated due to impairment of renal reclamation of biotin. Levels may also be increased from prolonged consumption of raw egg-whites (PMID: 16895887, 9523856, 15447901, 9176832)(OMIM: 210210, 253270, 600529, 253260, 246450, 210200, 238331)
Chemical_IUPAC_Name3-hydroxy-3-methyl-Butanoic acid
Chemical FormulaC5H10O3
Sample ConcentrationNot Available
Mass ValueNot Available
Mass Unitmg
ManufacturerQuattro_QQQ
AnalyzerTriple_Quad
DeliveryFlow_Injection
IonizationPositive
Predicted 1H NMR SpectrumDownload
Predicted 1H NMR PeaklistDownload
Predicted 13C NMR SpectrumDownload
Predicted 13C NMR PeaklistDownload
Sample ConcentrationNot Available
Mass ValueNot Available
Mass UnitNot Available
ManufacturerNot Available
FrequencyNot Available
1H NMR SpectrumNot Available
Sample ConcentrationNot Available
Mass ValueNot Available
Mass UnitNot Available
ManufacturerNot Available
FrequencyNot Available
13C NMR SpectrumNot Available
Low Energy Voltage10
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