• Gene id: 51004

Gene Summary

• Gene Symbol: COQ6   Gene   UCSC   Ensembl
• Aliases: CGI-10, CGI10, COQ10D6
• Gene name: coenzyme Q6, monooxygenase
• Alternate names: ubiquinone biosynthesis monooxygenase COQ6, mitochondrial, coenzyme Q10 monooxygenase 6, coenzyme Q6 homolog, monooxygenase,
• Gene location: 14q24.3 (73949933: 73963669)     Exons: 15     NC_000014.9
• Gene summary(Entrez): 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,
• OMIM: 610882

Protein Summary

• Protein general information: Q9Y2Z9  
  • Name: Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial (EC 1.14.13. ) (Coenzyme Q10 monooxygenase 6)
  • Length: 468  
  • Mass: 50870
  • Tissue specificity: Widely expressed. {ECO
• Sequence:

  MAARLVSRCGAVRAAPHSGPLVSWRRWSGASTDTVYDVVVSGGGLVGAAMACALGYDIHFHDKKILLLEAGPKKV
  LEKLSETYSNRVSSISPGSATLLSSFGAWDHICNMRYRAFRRMQVWDACSEALIMFDKDNLDDMGYIVENDVIMH
  ALTKQLEAVSDRVTVLYRSKAIRYTWPCPFPMADSSPWVHITLGDGSTFQTKLLIGADGHNSGVRQAVGIQNVSW
  NYDQSAVVATLHLSEATENNVAWQRFLPSGPIALLPLSDTLSSLVWSTSHEHAAELVSMDEEKFVDAVNSAFWSD
  ADHTDFIDTAGAMLQYAVSLLKPTKVSARQLPPSVARVDAKSRVLFPLGLGHAAEYVRPRVALIGDAAHRVHPLA
  GQGVNMGFGDISSLAHHLSTAAFNGKDLGSVSHLTGYETERQRHNTALLAATDLLKRLYSTSASPLVLLRTWGLQ
  ATNAVSPLKEQIMAFASK

• Structural information:
  • Interpro: IPR002938  IPR036188  IPR018168  IPR010971  IPR000689  
  • Prosite: PS01304
  • STRING: ENSP00000333946
• Other Databases: GeneCards:  COQ6  Malacards:  COQ6

Gene ontology

GO accession	Term name	Evidence code	Go category
GO:0005739	mitochondrion	IBA	cellular component
GO:0006744	ubiquinone biosynthetic process	IBA	biological process
GO:0016491	oxidoreductase activity	IBA	molecular function
GO:0004497	monooxygenase activity	IEA	molecular function
GO:0016709	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	IEA	molecular function
GO:0050660	flavin adenine dinucleotide binding	IEA	molecular function
GO:0055114	oxidation-reduction process	IEA	biological process
GO:0071949	FAD binding	IEA	molecular function
GO:0006744	ubiquinone biosynthetic process	IEA	biological process
GO:0005794	Golgi apparatus	IEA	cellular component
GO:0004497	monooxygenase activity	IEA	molecular function
GO:0042995	cell projection	IEA	cellular component
GO:0055114	oxidation-reduction process	IEA	biological process
GO:0005743	mitochondrial inner membrane	IEA	cellular component
GO:0016491	oxidoreductase activity	IEA	molecular function
GO:0016020	membrane	IEA	cellular component
GO:0005739	mitochondrion	IEA	cellular component
GO:0006744	ubiquinone biosynthetic process	IEA	biological process
GO:0005739	mitochondrion	IDA	cellular component
GO:0005515	protein binding	IPI	molecular function
GO:0005515	protein binding	IPI	molecular function
GO:0005515	protein binding	IPI	molecular function
GO:0005515	protein binding	IPI	molecular function
GO:0042995	cell projection	IEA	cellular component
GO:0005794	Golgi apparatus	IEA	cellular component
GO:0005743	mitochondrial inner membrane	IEA	cellular component
GO:0016712	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	IEA	molecular function
GO:0005794	Golgi apparatus	IEA	cellular component
GO:0042995	cell projection	IEA	cellular component
GO:0006744	ubiquinone biosynthetic process	IEA	biological process
GO:0031314	extrinsic component of mitochondrial inner membrane	IEA	cellular component
GO:0006744	ubiquinone biosynthetic process	IEA	biological process

KEGG pathways

Pathway id	Pathway name
hsa01100	Metabolic pathways
hsa00130	Ubiquinone and other terpenoid-quinone biosynthesis

Diseases

Associated diseases	References
Coenzyme Q10 deficiency	KEGG:H00999
Coenzyme Q10 deficiency	KEGG:H00999
Spermatogenic defects	MIK: 31037746

PubMed references

PMID	Condition	Mutation	Ethnicity	Population details	Infertility_type	Associated_genes
31037746	Spermatogenic defects			28 men with azoospermia	Male infertility	Microarray