2-Methyl-3-hydroxybutyryl-CoA

2-methyl-3-hydroxybutyryl-CoA.svg 250px S-[2-[3-[[4-[[[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-phosphonooxyoxolan-2-yl]methoxy-hydroxyphosphoryl]oxy-hydroxyphosphoryl]oxy-2-hydroxy-3,3-dimethylbutanoyl]amino]propanoylamino]ethyl] (2S,3S)-3-hydroxy-2-methylbutanethioate 165613 389295 C04405 1S/C26H44N7O18P3S/c1-13(14(2)34)25(39)55-8-7-28-16(35)5-6-29-23(38)20(37)26(3,4)10-48-54(45,46)51-53(43,44)47-9-15-19(50-52(40,41)42)18(36)24(49-15)33-12-32-17-21(27)30-11-31-22(17)33/h11-15,18-20,24,34,36-37H,5-10H2,1-4H3,(H,28,35)(H,29,38)(H,43,44)(H,45,46)(H2,27,30,31)(H2,40,41,42)/t13-,14-,15+,18+,19+,20?,24+/m0/s1 PEKYNTFSOBAABV-SYASONGASA-N 440326 C[C@@H]([C@H](C)O)C(=O)SCCNC(=O)CCNC(=O)C(C(C)(C)COP(=O)(O)OP(=O)(O)OC[C@@H]1[C@H]([C@H]([C@@H](O1)N2C=NC3=C(N=CN=C32)N)O)OP(=O)(O)O)O C26H44N7O18P3S 867.7 g/mol 2-Methyl-3-hydroxybutyryl-CoA (2M3HBAJanuary 2005 2-Methyl-3-Hydroxybutyryl-CoA Dehydrogenase (MHBD) Deficiency: An X-linked Inborn Error of Isoleucine Metabolism that May Mimic a Mitochondrial Disease https://www.nature.com/articles/pr2005690 Pediatric Research en 58 3 488–491 10.1203/01.pdr.0000176916.94328.cd 1530-0447 Perez-Cerda Celia García-Villoria Judit Ofman Rob Sala Pedro Ruiz Merinero Begoña Ramos Julio García-Silva Maria Teresa Beseler Beatriz Dalmau Jaime Wanders Ronald J A. Ugarte Magdalena Ribes Antonia 16148061 subscription ) is an intermediate in isoleucine catabolism. It is a substrate for the enzyme 3-hydroxy-2-methylbutyryl-CoA dehydrogenase (1.1.1.178) which converts it to 2-methylacetoacetyl-CoA:1.1.1.178 60% frameless|class=skin-invert-image 2-methyl-3-hydroxybutyryl-CoA + NAD+ reversible H+ H+ class=skin-invert-image 2-methylacetoacetyl-CoA + NADH References Category:Thioesters of coenzyme A