COQ2 ligates all-E-10PrP2 to PHB
PHB + all-E-10PrP2 → DHB + PPi
- Reactome
- R-HSA-2162192
- Compartment
- mitochondrial inner membrane · mitochondrial matrix
- Rhea
- RHEA:44564, RHEA:44565
Inputs
- PHBCHEBI:17879, C7H5O3, mitochondrial matrix
- all-E-10PrP2CHEBI:60721, C50H81O7P2, mitochondrial inner membrane
Outputs
- DHBCHEBI:84503, C57H85O3, mitochondrial inner membrane
- PPiCHEBI:33019, HO7P2, mitochondrial matrix
Catalysed by
- 4-hydroxybenzoate polyprenyltransferase activity of COQ2 [mitochondrial inner membrane]EC 2.5.1.39; UniProt Q96H96
Literature
- Isolation and functional expression of human COQ2, a gene encoding a polyprenyl transferase involved in the synthesis of CoQ Biochem J, 2004
- Investigation of coenzyme Q biosynthesis in human fibroblast and HepG2 cells J Biochem Biophys Methods, 2008
- Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis Hum Mol Genet, 2007