Reactome | COQ3 methylates DeMQ10H2

COQ3 methylates DeMQ10H2

Stable Identifier

R-HSA-2162186

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

DeMQ10H2 is methylated to Q10H2 by COQ3

ReviewStatus

5/5

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Mitochondrial COQ3 is an O-methyltransferase required in the reaction to convert 3-demethylubiquinol-10 (DeMQ10H2) to ubiquinol-10 (Q10H2) (Jonassen & Clarke 2000). Nicoll et al. (2024) confirmed the activity of COQ3 in an in vitro assay that comprised also COQ7 and COQ9, as it was not possible to study the methylation step on its own due to the instability of the short chain analog of DeMQ10H2.

Literature References

PubMed ID	Title	Journal	Year
38425362	In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis Gottinger, A, Mascotti, ML, Alvigini, L, Mattevi, A, Nicoll, CR, Cecchini, D, Corana, F, Mannucci, B	Nat Catal	2024
26251450	Structural and biochemical studies reveal UbiG/Coq3 as a class of novel membrane-binding proteins Zhang, X, Zhu, Y, Niu, L, Wu, B, Wang, J, Li, X, Fan, X, Teng, M	Biochem J	2015
10777520	Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis Jonassen, T, Clarke, CF	J Biol Chem	2000