3-ketopristanoyl-CoA + CoASH => 4,8,12-trimethyltridecanoyl-CoA + propionyl-CoA
3-ketopristanoyl-CoA + CoA-SH → 4,8,12-trimethyltridecanoyl-CoA + propionyl CoA
- Reactome
- R-HSA-390224
- Compartment
- peroxisomal matrix
- Rhea
- RHEA:10410
Inputs
- 3-ketopristanoyl-CoACHEBI:57291, C40H66N7O18P3S, peroxisomal matrix
- CoA-SHCHEBI:57287, C21H32N7O16P3S, peroxisomal matrix
Outputs
- 4,8,12-trimethyltridecanoyl-CoACHEBI:57351, C37H62N7O17P3S, peroxisomal matrix
- propionyl CoACHEBI:57392, C24H36N7O17P3S, peroxisomal matrix
Catalysed by
- propanoyl-CoA C-acyltransferase activity of SCP2-1 [peroxisomal matrix]EC 2.3.1.176; UniProt P22307
Literature
- Mutations in the gene encoding peroxisomal sterol carrier protein X (SCPx) cause leukencephalopathy with dystonia and motor neuropathy Am J Hum Genet, 2006
- Sterol carrier protein X (SCPx) is a peroxisomal branched-chain beta-ketothiolase specifically reacting with 3-oxo-pristanoyl-CoA: a new, unique role for SCPx in branched-chain fatty acid metabolism in peroxisomes Biochem Biophys Res Commun, 1997