2.0 2012-05-31 13:03:56 -0600 2015-06-03 15:53:41 -0600 ECMDB01052 M2MDB000234 (S)-3-Hydroxyisobutyryl-CoA (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). (S)-3-Hydroxyisobutyryl-CoA (S)-3-Hydroxyisobutyryl-Coenzyme A 3-Hydroxy-2-methylpropanoyl-CoA 3-Hydroxy-2-methylpropanoyl-Coenzyme A 3-Hydroxy-2-methylpropionyl-CoA 3-Hydroxy-2-methylpropionyl-Coenzyme A 3-Hydroxy-isobutyryl-CoA 3-Hydroxy-isobutyryl-coenzyme A 3-Hydroxyisobutyryl-CoA 3-Hydroxyisobutyryl-Coenzyme A C25H42N7O18P3S 853.623 853.151987801 {[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 [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 319440-43-2 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=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) WWEOGFZEFHPUAM-UHFFFAOYSA-N Solid Cytoplasm Periplasm logp -0.56 ALOGPS logs -2.31 ALOGPS solubility 4.14e+00 g/l ALOGPS logp -5.9 ChemAxon pka_strongest_acidic 0.82 ChemAxon pka_strongest_basic 4.01 ChemAxon iupac {[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 ChemAxon average_mass 853.623 ChemAxon mono_mass 853.151987801 ChemAxon 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 ChemAxon formula C25H42N7O18P3S ChemAxon inchi 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) ChemAxon inchikey WWEOGFZEFHPUAM-UHFFFAOYSA-N ChemAxon polar_surface_area 383.86 ChemAxon refractivity 183.18 ChemAxon polarizability 75.7 ChemAxon rotatable_bond_count 22 ChemAxon acceptor_count 18 ChemAxon donor_count 10 ChemAxon physiological_charge -4 ChemAxon formal_charge 0 ChemAxon Valine, leucine and isoleucine degradation ec00280 Specdb::EiMs 3763 Specdb::NmrOneD 146210 Specdb::NmrOneD 146211 Specdb::NmrOneD 146212 Specdb::NmrOneD 146213 Specdb::NmrOneD 146214 Specdb::NmrOneD 146215 Specdb::NmrOneD 146216 Specdb::NmrOneD 146217 Specdb::NmrOneD 146218 Specdb::NmrOneD 146219 Specdb::NmrOneD 146220 Specdb::NmrOneD 146221 Specdb::NmrOneD 146222 Specdb::NmrOneD 146223 Specdb::NmrOneD 146224 Specdb::NmrOneD 146225 Specdb::NmrOneD 146226 Specdb::NmrOneD 146227 Specdb::NmrOneD 146228 Specdb::NmrOneD 146229 Specdb::MsMs 23141 Specdb::MsMs 23142 Specdb::MsMs 23143 Specdb::MsMs 29939 Specdb::MsMs 29940 Specdb::MsMs 29941 Specdb::MsMs 2409898 Specdb::MsMs 2409899 Specdb::MsMs 2409900 Specdb::MsMs 2555650 Specdb::MsMs 2555651 Specdb::MsMs 2555652 HMDB01052 88 86 C06000 28259 3-HYDROXY-ISOBUTYRYL-COA Kanehisa, M., Goto, S., Sato, Y., Furumichi, M., Tanabe, M. (2012). "KEGG for integration and interpretation of large-scale molecular data sets." Nucleic Acids Res 40:D109-D114. 22080510 Loupatty, F. J., Clayton, P. T., Ruiter, J. P., Ofman, R., Ijlst, L., Brown, G. K., Thorburn, D. R., Harris, R. A., Duran, M., Desousa, C., Krywawych, S., Heales, S. J., Wanders, R. J. (2007). "Mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration." Am J Hum Genet 80:195-199. 17160907 Lehnert W, Sass JO: Glutaconyl-CoA is the main toxic agent in glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I). Med Hypotheses. 2005;65(2):330-3. 15922108 Hawes, John W.; Harper, Edwin T. Synthesis of methacrylyl-CoA and (R)- and (S)-3-hydroxyisobutyryl-CoA. Methods in Enzymology (2000), 324(Branched-Chain Amino Acids), 73-79. Fatty acid oxidation complex subunit alpha P21177 FADB_ECOLI fadB http://ecmdb.ca/proteins/P21177.xml Probable enoyl-CoA hydratase paaF P76082 PAAF_ECOLI paaF http://ecmdb.ca/proteins/P76082.xml Fatty acid oxidation complex subunit alpha_ P77399 FADJ_ECOLI fadJ http://ecmdb.ca/proteins/P77399.xml Fatty acid oxidation complex subunit alpha P21177 FADB_ECOLI fadB http://ecmdb.ca/proteins/P21177.xml Methacrylyl-CoA + Water <> (S)-3-Hydroxyisobutyryl-CoA R04224