2-Methyl-3-hydroxybutyryl-CoA

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2-Methyl-3-hydroxybutyryl-CoA
2-methyl-3-hydroxybutyryl-CoA.svg
Names
IUPAC name
S-[2-[3-[[4-[[[(2R,3S,4R,5R)-{{#parsoidfragment:1}} (2S,3S)-3-hydroxy-2-methylbutanethioate
Identifiers
3D model (JSmol)
ChEBI
ChemSpider
KEGG
PubChem CID
  • InChI=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
    Key: PEKYNTFSOBAABV-SYASONGASA-N
  • 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
Properties
C26H44N7O18P3S
Molar mass 867.7 g/mol
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).

2-Methyl-3-hydroxybutyryl-CoA (2M3HBA [1] ) is an intermediate in isoleucine catabolism.

It is a substrate for the enzyme 3-hydroxy-2-methylbutyryl-CoA dehydrogenase (EC 1.1.1.178) which converts it to 2-methylacetoacetyl-CoA: [2]

(S)-3-Hydroxy-2-methylbutyryl-CoA.svg
2-methyl-3-hydroxybutyryl-CoA
+ NAD+
 
 
 
H+
2-Methyl-3-hydroxybutyryl-CoA
 
H+
 
2-Methylacetoacetyl-CoA.svg
2-methylacetoacetyl-CoA
+ NADH
 

References

  1. ↑ 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 (January 2005). "2-Methyl-3-Hydroxybutyryl-CoA Dehydrogenase (MHBD) Deficiency: An X-linked Inborn Error of Isoleucine Metabolism that May Mimic a Mitochondrial Disease". Pediatric Research . 58 (3): 488–491. doi:10.1203/01.pdr.0000176916.94328.cd. ISSN   1530-0447. PMID   16148061.
  2. ↑ Enzyme 1.1.1.178 at KEGG Pathway Database.
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