Accession Number | HMDB00396 |
Common_Name | 2-Ethylhydracrylic acid |
Description | Isolated excretion of 2-Ethylhydracrylic acid (2-methylbutyrylglycine; 2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), a defect in the proximal pathway of L-isoleucine oxidation (PMID 15615815)
Normal human urine contains small amounts (less than 4 mg/g of creatinine) of 2-ethylhydracrylic acid. Urinary excretion of 2-ethylhydracrylic acid is variably increased in defects of isoleucine oxidation at distal steps in the catabolic pathway (3-oxoacyl-CoA thiolase deficiency and methylmalonyl-CoA mutase deficiency) and is diminished when proximal steps of the oxidative pathway are blocked as in branched-chain oxo acid decarboxylase deficiency ('maple-syrup-urine' disease).
(PMID: 1016232) |
Chemical_IUPAC_Name | 2-(hydroxymethyl)-Butanoic acid |
Chemical Formula | C5H10O3 |
Sample Concentration | Not Available |
Mass Value | Not Available |
Mass Unit | Not Available |
Manufacturer | Not Available |
Analyzer | Not Available |
Delivery | Not Available |
Ionization | Not Available |
Predicted 1H NMR Spectrum | Download |
Predicted 1H NMR Peaklist | Download |
Predicted 13C NMR Spectrum | Download |
Predicted 13C NMR Peaklist | Download |
Sample Concentration | Not Available |
Mass Value | Not Available |
Mass Unit | Not Available |
Manufacturer | Not Available |
Frequency | Not Available |
1H NMR Spectrum | Not Available |
Sample Concentration | Not Available |
Mass Value | Not Available |
Mass Unit | Not Available |
Manufacturer | Not Available |
Frequency | Not Available |
13C NMR Spectrum | Not Available |
Low Energy Voltage | Not Available |
Low Energy Spectrum | Not Available |
Low Energy Peaklist | Not Available |
Medium Energy Spectrum | Not Available |
Hight Energy Spectrum | Not Available |