COQ5

coenzyme Q5, methyltransferase

Summary

Enables 2-methoxy-6-polyprenyl-1,4-benzoquinol methyltransferase activity. Involved in methylation and ubiquinone biosynthetic process. Located in mitochondrial inner membrane and mitochondrial matrix. Part of ubiquinone biosynthesis complex. Implicated in primary coenzyme Q10 deficiency 9. [provided by Alliance of Genome Resources, Jul 2025]

Known Variants34 total

rsidPosition (GRCh37)AllelesClassClinVar
rs91567036312:120,941,637C/Tuncertain significance
rs14350684212:120,941,785C/Tbenign
rs75341541312:120,941,813T/Auncertain significance
rs135101375112:120,941,890C/Tlikely benign
rs14068779912:120,942,712A/Tuncertain significance
rs76945802112:120,942,746C/Tuncertain significance
rs167177012:120,946,568A/G
rs186905885512:120,947,890G/Tuncertain significance
rs77842099712:120,947,905T/Auncertain significance
rs7457859412:120,947,933G/Cbenign
rs74898109612:120,954,424T/Guncertain significance
rs52996571112:120,954,447C/Tlikely benign
rs54335599012:120,954,448G/Auncertain significance
rs130761633912:120,954,453C/Tuncertain significance
rs75957574912:120,954,463A/Glikely benign
rs186938638512:120,954,483T/Guncertain significance
rs14625468312:120,954,549T/Clikely benign
rs37766817412:120,954,591C/Tconflicting classifications of pathogenicity
rs13881419512:120,960,009G/Alikely benign
rs117790675912:120,960,036A/Clikely benign
rs20122189812:120,960,055A/Glikely benign
rs50333512:120,960,057T/Cbenign
rs14278432812:120,960,102A/Glikely benign
rs124142591112:120,960,108A/Cuncertain significance
rs250058394312:120,960,118T/Cuncertain significance
rs116769712:120,960,341A/Tdownstream gene variant
rs124272590112:120,966,764C/Tuncertain significance
rs76369063812:120,966,789C/Alikely benign
rs19975257812:120,966,845G/Auncertain significance
rs77615641012:120,966,859A/Guncertain significance
rs75737318912:120,966,914T/Guncertain significance
rs14004335712:120,966,929T/Clikely benign
rs52763022512:120,966,932C/Tlikely benign
rs56391255512:120,966,935G/Auncertain significance

Gene information from NCBI Gene. Variant classifications from ClinVar.