acetoacetyl-CoA+acetyl-CoA => HMG-CoA + CoASH
ACA-CoA + Ac-CoA → HMG CoA + CoA-SH
- Reactome
- R-HSA-73918
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:10189
Inputs
- ACA-CoACHEBI:57286, C25H36N7O18P3S, mitochondrial matrix
- Ac-CoACHEBI:57288, C23H34N7O17P3S, mitochondrial matrix
Outputs
- HMG CoACHEBI:15467, C27H44N7O20P3S, mitochondrial matrix
- CoA-SHCHEBI:57287, C21H32N7O16P3S, mitochondrial matrix
Catalysed by
- hydroxymethylglutaryl-CoA synthase activity of HMGCS2 [mitochondrial matrix]EC 2.3.3.10; UniProt P54868
Literature
- Genetic basis of mitochondrial HMG-CoA synthase deficiency Hum Genet, 2001