5-demethoxyubiquinone hydroxylase, mitochondrial

Definition:

Catalyzes the hydroxylation of 2-polyprenyl-3-methyl-6-methoxy-1,4-benzoquinol (DMQH2) during ubiquinone biosynthesis. Has also a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides. Involved in lifespan determination in a ubiquinone-independent manner.

References:

[1]. Richard M Monaghan, et al. A nuclear role for the respiratory enzyme CLK-1 in regulating mitochondrial stress responses and longevity. Nat Cell Biol. 2015 Jun;17(6):782-92. [Content Brief]

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