Accession Number | HMDB01052 |
Common_Name | (S)-3-Hydroxyisobutyryl-CoA |
Description | (S)-3-Hydroxyisobutyryl-CoA is s metabolite of 3-hydroxyisobutyryl-CoA hydrolase (EC 3.1.2.4 ) during beta-alanine metabolism (KEGG 00410), propanoate metabolism (KEGG 00640), and valine, leucine and isoleucine degradation (KEGG 00280). Deficiencies of this enzyme in valine degradation can result in hypotonia, poor feeding, motor delay, and subsequent neurological regression in infancy, episodes of ketoacidosis and Leigh-like changes in the basal ganglia on a magnetic resonance imaging scan (PMID 17160907). |
Chemical_IUPAC_Name | S-[2-[3-[[4-[[[5-(6-aminopurin-9-yl)-4-hydroxy-3-phosphonooxyoxolan-2-yl]methoxy-hydroxyphosphoryl]oxy-hydroxyphosphoryl]oxy-2-hydroxy-3,3-dimethylbutanoyl]amino]propanoylamino]ethyl] 3-hydroxy-2-methylpropanethioate |
Chemical Formula | C25H42N7O18P3S |
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 |