Involved in ubiquinone biosynthetic process. Predicted to be extrinsic component of mitochondrial inner membrane. Predicted to be active in mitochondrion. Human ortholog(s) of this gene implicated in primary coenzyme Q10 deficiency 7 and spastic ataxia. Orthologous to human COQ4 (coenzyme Q4).
WB Description
coq-4 encodes an ortholog of S. cerevisiae COQ4; while COQ-4 is conservedbetween eukaryotes and bacteria, its biochemical function is unknown; byorthology, COQ-4 is predicted to peripherally associate with the matrixface of the mitochondrial inner membrane, in a complex with COQ-3 (andperhaps COQ-6), and to be required to maintain a steady-state level ofCLK-1/COQ-7 protein; COQ-4 is required for ubiquinone (coenzyme Q9)biosynthesis and for normally short lifespan; coq-4(RNAi) animals havereduced levels of coenzyme Q9 and superoxide, and have abnormally longlifespans.