COQ3 methylates DeMQ10H2
DeMQ10H2 + AdoMet → CoQH2 + AdoHcy + H+
- Reactome
- R-HSA-2162186
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:44412, RHEA:44413
Inputs
- DeMQ10H2CHEBI:64182, C58H90O4, mitochondrial inner membrane
- AdoMetCHEBI:59789, C15H23N6O5S, mitochondrial matrix
Outputs
- CoQH2CHEBI:64183, C59H92O4, mitochondrial inner membrane
- AdoHcyCHEBI:57856, C14H20N6O5S, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
Catalysed by
- 3-demethylubiquinol 3-O-methyltransferase activity of COQ3:COQ4:COQ5:COQ6:COQ7:COQ9 [mitochondrial inner membrane]EC 2.1.1.64
Literature
- Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis J Biol Chem, 2000
- Structural and biochemical studies reveal UbiG/Coq3 as a class of novel membrane-binding proteins Biochem J, 2015
- In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis Nat Catal, 2024