Accession Number | HMDB00339 |
Common_Name | 2-Methylbutyrylglycine |
Description | Isolated excretion of 2-methylbutyrylglycine (2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase deficiency (PubMedID 15615815)
A number of previously unrecognized abnormal metabolites have been identified and quantitated in the urine of a patient with an inherited deficiency of propionyl-CoA carboxylase. These included the isoleucine metabolites 2-methyl-3-hydroxybutyric acid and 2-methylacetoacetic acid. These isomers 3-hydroxyvaleric acid and 3-oxovaleric acid were found, which may be products of the condensation of propionyl-CoA with acetyl-CoA catalyzed by 3-oxoacyl-CoA thiolases. Following a load of isoleucine, 2-methylbutyrylglycine was identified. This metabolite has not previously been observed in man. (PMID: 630060) |
Chemical_IUPAC_Name | N-(2-methyl-1-oxobutyl)-Glycine |
Chemical Formula | C7H13NO3 |
Sample Concentration | 1 |
Mass Value | Not Available |
Mass Unit | mg |
Manufacturer | Quattro_QQQ |
Analyzer | Triple_Quad |
Delivery | Flow_Injection |
Ionization | Positive |
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 |  |
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 | 10 |
Low Energy Spectrum | Download |
Low Energy Peaklist | Download |
Medium Energy Spectrum | Download |
High Energy Spectrum | Download |