Reactome | COQ5 methylates MDMQ10H2

COQ5 methylates MDMQ10H2

Stable Identifier

R-HSA-2162188

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

MDMQ10H2 is methylated to DMQ10H2 by COQ5

ReviewStatus

5/5

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

2-methoxy-6-polyprenyl-1,4-benzoquinol methylase (COQ5) catalyses the C-methyltransferase conversion of 2-methoxy-6-decaprenyl-1,4-benzoquinol (MDMQ10H2) to 6-methoxy-3-methyl-2-decaprenyl-1,4-benzoquinol (DMQ10H2). The monomer, while localized in the mitochondrial matrix, is part of a multimeric complex associated to the mitochondrial inner membrane (Chen et al., 2013; Nguyen et al., 2015; Yen et al., 2016). The activity was confirmed with an in vitro assay using a short chain substrate analog and a resurrected ancestral COQ5 (Nicoll et al., 2024). This reaction was first studied in yeast (Barkovich et al. 1997).

Literature References

PubMed ID	Title	Journal	Year
27155576	Disruption of the human COQ5-containing protein complex is associated with diminished coenzyme Q10 levels under two different conditions of mitochondrial energy deficiency Yen, HC, Liu, YC, Kan, CC, Wei, HJ, Lee, SH, Wei, YH, Feng, YH, Chen, CW, Huang, CC	Biochim Biophys Acta	2016
25152161	Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q10 biosynthesis Nguyen, TP, Casarin, A, Desbats, MA, Doimo, M, Trevisson, E, Santos-Ocaña, C, Navas, P, Clarke, CF, Salviati, L	Biochim Biophys Acta	2014
38425362	In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis Nicoll, CR, Alvigini, L, Gottinger, A, Cecchini, D, Mannucci, B, Corana, F, Mascotti, ML, Mattevi, A	Nat Catal	2024
9083049	Characterization of the COQ5 gene from Saccharomyces cerevisiae. Evidence for a C-methyltransferase in ubiquinone biosynthesis Barkovich, RJ, Shtanko, A, Shepherd, JA, Lee, PT, Myles, DC, Tzagoloff, A, Clarke, CF	J Biol Chem	1997
23354120	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 Chen, SW, Liu, CC, Yen, HC	Mitochondrion	2013