Record Information
Version1.0
StatusDetected and Quantified
Creation Date2021-11-17 23:31:27 UTC
Update Date2024-04-30 19:37:29 UTC
Metabolite IDMMDBc0029537
Metabolite Identification
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 ).
Structure
Synonyms
ValueSource
(S)-3-Hydroxyisobutyryl-coenzyme AHMDB
3-Hydroxy-2-methylpropanoyl-CoAHMDB
3-Hydroxy-2-methylpropanoyl-coenzyme AHMDB
3-Hydroxy-2-methylpropionyl-CoAHMDB
3-Hydroxy-2-methylpropionyl-coenzyme AHMDB
3-Hydroxy-isobutyryl-CoAHMDB
3-Hydroxy-isobutyryl-coenzyme AHMDB
3-Hydroxyisobutyryl-CoAHMDB
3-Hydroxyisobutyryl-coenzyme AHMDB
Molecular FormulaC25H42N7O18P3S
Average Mass853.623
Monoisotopic Mass853.151987801
IUPAC Name{[5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxy-2-methylpropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid
Traditional Name[5-(6-aminopurin-9-yl)-4-hydroxy-2-({[hydroxy([hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxy-2-methylpropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxyphosphonic acid
CAS Registry Number319440-43-2
SMILES
CC(CO)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12
InChI Identifier
InChI=1S/C25H42N7O18P3S/c1-13(8-33)24(38)54-7-6-27-15(34)4-5-28-22(37)19(36)25(2,3)10-47-53(44,45)50-52(42,43)46-9-14-18(49-51(39,40)41)17(35)23(48-14)32-12-31-16-20(26)29-11-30-21(16)32/h11-14,17-19,23,33,35-36H,4-10H2,1-3H3,(H,27,34)(H,28,37)(H,42,43)(H,44,45)(H2,26,29,30)(H2,39,40,41)
InChI KeyWWEOGFZEFHPUAM-UHFFFAOYSA-N