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Gene

COQ6

coenzyme Q6, monooxygenase

Summary

Cytogenetic location
14q24.3
Coordinates
Chromosome 14: 73,950,302–73,963,670 (GRCh38)
Exons
12 exons across 13.4 kb
Protein
468 amino acids
Inheritance
Autosomal recessive
Also known as
CGI-10
MANE Select transcript
ENST00000334571.7NM_182476.3
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Reference genomeGRCh38

Positions on gene and variant pages follow this choice, in this browser.

Curated reports relevant to this gene

  • Nephrotic Syndrome

    • Nephrosis

      Pathological processes of the KIDNEY without inflammatory or neoplastic components. Nephrosis may be a primary disorder or secondary complication of other diseases. It is characterized by the NEPHROTIC SYNDROME indicating the presence of PROTEINURIA and HYPOALBUMINEMIA with accompanying EDEMA.

      • 1 GenCC submitter rates it definitive or strong

  • Primary Mitochondrial Disease

    • Coenzyme Q10 DeficiencyAutosomal recessive

      Caused by variants in genes needed to make coenzyme Q10, such as COQ2, COQ8A and PDSS2, which can cause kidney disease, balance problems, muscle weakness or brain disease.

      • ClinGen rates the link definitive
      • 2 GenCC submitters rate it definitive or strong
      What knowing changes

      Knowing is actionable: primary coenzyme Q10 deficiency can respond to high-dose coenzyme Q10 supplements, especially when started early.

Inheritance

Autosomal recessive
It takes a changed copy from each parent. With one, you are a carrier and usually unaffected; when both parents are carriers, each child has a one-in-four chance of inheriting two.

How COQ6 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 14, with a bracket where COQ6 sits. COQ6 and 23 genes near it on chromosome 14, from 73,456,986 to 74,456,985.Chromosome 1414q24.3p13p12p11.2p11.1q11.1q11.2q12q13.1q13.2q13.3q21.1q21.2q21.3q22.1q22.2q22.3q23.1q23.2q23.3q24.1q24.2q24.3q31.1q31.2q31.3q32.11q32.12q32.13q32.2q32.31q32.32q32.3373.6 Mb73.8 Mb74 Mb74.2 Mb74.4 MbNUMB: 73,274,146–73,466,167, reverse strandNUMBHEATR4: 73,478,484–73,558,947, reverse strandHEATR4RIOX1: 73,490,933–73,520,134, forward strandRIOX1ACOT1: 73,537,122–73,543,797, forward strandACOT1ACOT2: 73,567,620–73,588,885, forward strandACOT2ACOT4: 73,591,660–73,595,766, forward strandACOT4ACOT6: 73,610,945–73,619,888, forward strandACOT6DNAL1: 73,644,875–73,703,732, forward strandDNAL1PNMA1: 73,711,783–73,714,384, reverse strandPNMA1MIDEAS: 73,715,122–73,790,285, reverse strandMIDEASPTGR2: 73,851,792–73,885,726, forward strandPTGR2: 73,851,971–73,932,278, forward strandZNF410: 73,886,201–73,932,521, forward strandZNF410FAM161B: 73,931,501–73,950,414, reverse strandFAM161BCOQ6: 73,950,302–73,963,670, forward strandCOQ6ENTPD5: 73,956,688–74,019,399, reverse strandENTPD5BBOF1: 74,019,341–74,082,863, forward strandBBOF1ALDH6A1: 74,055,997–74,084,492, reverse strandALDH6A1LIN52: 74,084,943–74,201,493, forward strandLIN52VSX2: 74,239,449–74,262,738, forward strandVSX2ABCD4: 74,285,269–74,303,068, reverse strandABCD4VRTN: 74,303,069–74,360,011, forward strandVRTNSYNDIG1L: 74,405,894–74,426,325, reverse strandSYNDIG1LNPC2: 74,427,119–74,494,177, reverse strandNPC2Diagram of COQ6: 12 exons on the forward strand of chromosome 14Exon 1: 2 known disease-causing sitesExon 2: 3 known disease-causing sitesExon 3: 2 known disease-causing sitesExon 5: 1 known disease-causing siteExon 6: 1 known disease-causing siteExon 7: 1 known disease-causing siteExon 8: 1 known disease-causing siteExon 9: 6 known disease-causing sitesExon 10: 1 known disease-causing siteExon 11: 1 known disease-causing siteExon 1: 73,950,302–73,950,495, 194 basesExon 2: 73,953,435–73,953,569, 135 basesExon 3: 73,955,451–73,955,509, 59 basesExon 4: 73,955,805–73,955,928, 124 basesExon 5: 73,958,147–73,958,277, 131 basesExon 6: 73,958,971–73,959,078, 108 basesExon 7: 73,959,162–73,959,224, 63 basesExon 8: 73,959,415–73,959,522, 108 basesExon 9: 73,961,173–73,961,375, 203 basesExon 10: 73,961,455–73,961,570, 116 basesExon 11: 73,961,737–73,961,903, 167 basesExon 12: 73,962,970–73,963,670, 701 bases1246810125′3′

Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.

Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).

Nearby genes from Ensembl release 116, on GRCh38.

Exons make up 2.1 kb of the gene's 13.4 kb; 1,407 of those bases code for the protein.

How a genome test reads COQ6

20

positions in COQ6 where ClinVar classes a change as pathogenic or likely pathogenic

None of them falls in a region short reads find hard.

A whole genome covers all 13.4 kb of COQ6, introns included, not only the 2.1 kb of exons.

Where this comes from

  • HGNC: the gene's symbol, name, other symbols and location.
  • MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
  • GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
  • The ACMG secondary findings list, SF v3.2.
  • ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
  • The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
  • Data release 2026.10.16-c6eea6bf.

Cite this page

APA

Dante Labs. (n.d.). COQ6 gene: coenzyme Q6, monooxygenase. Retrieved 16 October 2026, from https://dantelabs.com/genes/coq6

Vancouver

Dante Labs. COQ6 gene: coenzyme Q6, monooxygenase [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/coq6

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