Flavin-dependent monooxygenase involved in ubiquinone biosynthesis; responsible for hydroxylation at position C5 and deamination at C4 during ubiquinone (Coenzyme Q) biosynthesis; localizes to matrix face of mitochondrial inner membrane in a large complex with other ubiquinone biosynthetic enzymes; human homolog COQ6 can complement yeast null mutant and is implicated in steroid-resistant nephrotic syndrome (SRNS) [Source:SGD;Acc:S000003487]
Chromosome VII: 1,002,523-1,003,962 reverse strand.
R64-1-1:BK006941.2
This gene has 1 transcript (splice variant), 326 orthologues, 1 paralogue and is a member of 2 Ensembl protein families.