COQ6

coenzyme Q6, monooxygenase | CGI-10

The protein encoded by this gene belongs to the ubiH/COQ6 family. It is an evolutionarily conserved monooxygenase required for the biosynthesis of coenzyme Q10 (or ubiquinone), which is an essential component of the mitochondrial electron transport chain, and one of the most potent lipophilic antioxidants implicated in the protection of cell damage by reactive oxygen species. Knockdown of this gene in mouse and zebrafish results in decreased growth due to increased apoptosis. Mutations in this gene are associated with autosomal recessive coenzyme Q10 deficiency-6 (COQ10D6), which manifests as nephrotic syndrome with sensorineural deafness. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jun 2012]

Biological processes 25 terms

2-methoxy-6-polyprenolphenol 4-hydroxylase activity (GO:0120538)2-methoxy-6-polyprenolphenol 4-hydroxylase activity (GO:0120538)4-hydroxy-3-all-trans-polyprenylbenzoate oxygenase activity (GO:0106364)4-hydroxy-3-all-trans-polyprenylbenzoate oxygenase activity (GO:0106364)FAD binding (GO:0071949)Golgi apparatus (GO:0005794)extrinsic component of mitochondrial inner membrane (GO:0031314)extrinsic component of mitochondrial inner membrane (GO:0031314)flavin adenine dinucleotide binding (GO:0050660)mitochondrial inner membrane (GO:0005743)mitochondrial inner membrane (GO:0005743)mitochondrial inner membrane (GO:0005743)mitochondrion (GO:0005739)mitochondrion (GO:0005739)mitochondrion (GO:0005739)monooxygenase activity (GO:0004497)oxidoreductase activity (GO:0016491)oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen (GO:0016705)oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen (GO:0016709)oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen (GO:0016712)protein binding (GO:0005515)ubiquinone biosynthetic process (GO:0006744)ubiquinone biosynthetic process (GO:0006744)ubiquinone biosynthetic process (GO:0006744)ubiquinone biosynthetic process (GO:0006744)

Expression (TPM)

TFs regulating COQ6 0 TFs

Transcription factors with Perturb-seq knockdown data for COQ6. The Binding column indicates whether any binding evidence exists for this TF–gene pair (ChIP-seq or motif footprint peaks). The Mean coef is the average Perturb-seq regression coefficient across active gRNAs (positive = COQ6 upregulated upon KD; negative = downregulated). The Outlier column indicates whether this gene is in the top or bottom 5% of all TF knockdown effects.

TF	Mean coef	Binding	Outlier	TF→Gene link

Rows:

Elements linked to COQ6

Open chromatin peaks (ATAC-seq) in the genomic neighbourhood of COQ6, linked by TSS proximity or chromatin conformation (Multiome / HiCAR). Each element overlaps at least one TF ChIP-seq binding site — the TFs column shows how many distinct TFs bind that element.

Element	Dist. to TSS	Link type	TFs
chr14:73,713,545–73,714,769	236.0 kb	Distal (>10kb) Multiome	759
chr14:73,718,770–73,719,334	231.2 kb	Distal (>10kb) Multiome	138
chr14:73,759,247–73,760,633	190.0 kb	Distal (>10kb) Multiome	912
chr14:73,771,644–73,772,269	178.3 kb	Distal (>10kb) Multiome	846
chr14:73,785,281–73,785,818	164.7 kb	Distal (>10kb) Multiome	458
chr14:73,786,685–73,787,517	163.1 kb	Distal (>10kb) Multiome	497
chr14:73,851,491–73,852,843	98.4 kb	Distal (>10kb) Multiome	842
chr14:73,886,331–73,887,426	63.5 kb	Distal (>10kb) Multiome	963
chr14:73,949,767–73,950,750	67 bp	At TSS Multiome	932
chr14:74,018,639–74,019,694	69.0 kb	Distal (>10kb) Multiome	791
chr14:74,083,828–74,085,467	134.4 kb	Distal (>10kb) Multiome	824

Genome Browser

Genomic view of the COQ6 locus showing ATAC-seq accessibility and RNA-seq expression across the ESC → DE time course, together with TF binding peaks and element-to-TSS loop connections.