COQ5 methylates MDMQ10H2
MDMQ10H2 + AdoMet → DMQ10H2 + AdoHcy + H+
- Reactome
- R-HSA-2162188
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:44764, RHEA:44765
Inputs
- MDMQ10H2CHEBI:64180, C57H88O3, mitochondrial inner membrane
- AdoMetCHEBI:59789, C15H23N6O5S, mitochondrial matrix
Outputs
- DMQ10H2CHEBI:64181, C58H90O3, mitochondrial inner membrane
- AdoHcyCHEBI:57856, C14H20N6O5S, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
Catalysed by
- 2-methoxy-6-polyprenyl-1,4-benzoquinol methyltransferase activity of COQ3:COQ4:COQ5:COQ6:COQ7:COQ9 [mitochondrial inner membrane]EC 2.1.1.201
Literature
- Characterization of the COQ5 gene from Saccharomyces cerevisiae. Evidence for a C-methyltransferase in ubiquinone biosynthesis J Biol Chem, 1997
- Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q10 biosynthesis Biochim Biophys Acta, 2014
- Disruption of the human COQ5-containing protein complex is associated with diminished coenzyme Q10 levels under two different conditions of mitochondrial energy deficiency Biochim Biophys Acta, 2016
- Detection of suppressed maturation of the human COQ5 protein in the mitochondria following mitochondrial uncoupling by an antibody recognizing both precursor and mature forms of COQ5 Mitochondrion, 2013
- In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis Nat Catal, 2024