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Aliases for COQ7 Gene

Aliases for COQ7 Gene

  • Coenzyme Q7, Hydroxylase 2 3
  • Ubiquinone Biosynthesis Monooxygenase COQ7 3 4
  • Coenzyme Q7 Homolog, Ubiquinone (Yeast) 2 5
  • 5-Demethoxyubiquinone Hydroxylase 2 3
  • Timing Protein Clk-1 Homolog 3 4
  • DMQ Hydroxylase 3 4
  • Ubiquinone Biosynthesis Protein COQ7 Homolog 3
  • Coenzyme Q Biosynthesis Protein 7 Homolog 3
  • COQ7 Coenzyme Q, 7 Homolog Ubiquinone 3
  • Coenzyme Q, 7 (Rat, Yeast) Homolog 2
  • Coenzyme Q7 Homolog, Ubiquinone 3
  • Placental Protein KG-20 3
  • EC 1.14.13.- 4
  • COQ10D8 3
  • CLK-1 3
  • CLK1 3
  • CAT5 3

External Ids for COQ7 Gene

Previous GeneCards Identifiers for COQ7 Gene

  • GC16P018569
  • GC16P019006
  • GC16P019045
  • GC16P018986
  • GC16P017616

Summaries for COQ7 Gene

Entrez Gene Summary for COQ7 Gene

  • The protein encoded by this gene is similar to a mitochondrial di-iron containing hydroxylase in Saccharomyces cerevisiae that is involved with ubiquinone biosynthesis. Mutations in the yeast gene lead to slower development and longer life span. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2010]

GeneCards Summary for COQ7 Gene

COQ7 (Coenzyme Q7, Hydroxylase) is a Protein Coding gene. Diseases associated with COQ7 include Coq7-Related Coenzyme Q10 Deficiency. Among its related pathways are Metabolism and Ubiquinol biosynthesis.

UniProtKB/Swiss-Prot for COQ7 Gene

  • Catalyzes the hydroxylation of 2-polyprenyl-3-methyl-6-methoxy-1,4-benzoquinol (DMQH2) during ubiquinone biosynthesis. Has also a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides. Involved in lifespan determination in a ubiquinone-independent manner.

Gene Wiki entry for COQ7 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for COQ7 Gene

Genomics for COQ7 Gene

Regulatory Elements for COQ7 Gene

Enhancers for COQ7 Gene
GeneHancer Identifier Score Enhancer Sources TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor Binding Sites within enhancer Other Gene Targets for Enhancer

Enhancers around COQ7 on UCSC Golden Path with GeneCards custom track

Promoters for COQ7 Gene
Ensembl Regulatory Elements (ENSRs) TSS Distance (bp) Size (bp) Binding Sites for Transcription Factors within promoters

ENSRs around COQ7 on UCSC Golden Path with GeneCards custom track

Genomic Location for COQ7 Gene

19,067,595 bp from pter
19,080,095 bp from pter
12,501 bases
Plus strand

Genomic View for COQ7 Gene

Genes around COQ7 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
COQ7 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for COQ7 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for COQ7 Gene

Proteins for COQ7 Gene

  • Protein details for COQ7 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    5-demethoxyubiquinone hydroxylase, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • B2RDA9
    • Q9BTT7
    • Q9H0T5
    • Q9UEW5
    • Q9UNR5

    Protein attributes for COQ7 Gene

    217 amino acids
    Molecular mass:
    24277 Da
    Name=Fe cation; Xref=ChEBI:CHEBI:24875;
    Quaternary structure:
    • Component of a multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9 (By similarity). Interacts with ADCK4 and COQ6 (PubMed:24270420). Interacts with COQ9 (PubMed:25339443).

    Alternative splice isoforms for COQ7 Gene


neXtProt entry for COQ7 Gene

Proteomics data for COQ7 Gene at MOPED

Post-translational modifications for COQ7 Gene

  • Ubiquitination at Lys 85
  • Modification sites at PhosphoSitePlus

No data available for DME Specific Peptides for COQ7 Gene

Domains & Families for COQ7 Gene

Suggested Antigen Peptide Sequences for COQ7 Gene

GenScript: Design optimal peptide antigens:

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the COQ7 family.
  • Belongs to the COQ7 family.
genes like me logo Genes that share domains with COQ7: view

No data available for Gene Families for COQ7 Gene

Function for COQ7 Gene

Molecular function for COQ7 Gene

GENATLAS Biochemistry:
coenzyme Q7 (rat,yeast) homolog C elegans longevity gene clk1 ortholog,expressed in heart and skeletal muscle,likely localized in the mitochondrial inner membrane
UniProtKB/Swiss-Prot Function:
Catalyzes the hydroxylation of 2-polyprenyl-3-methyl-6-methoxy-1,4-benzoquinol (DMQH2) during ubiquinone biosynthesis. Has also a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides. Involved in lifespan determination in a ubiquinone-independent manner.

Enzyme Numbers (IUBMB) for COQ7 Gene

genes like me logo Genes that share phenotypes with COQ7: view

Animal Models for COQ7 Gene

MGI Knock Outs for COQ7:

Animal Model Products

No data available for Gene Ontology (GO) - Molecular Function , Human Phenotype Ontology , Transcription Factor Targets and HOMER Transcription for COQ7 Gene

Localization for COQ7 Gene

Subcellular locations from UniProtKB/Swiss-Prot for COQ7 Gene

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Subcellular locations from

Jensen Localization Image for COQ7 Gene COMPARTMENTS Subcellular localization image for COQ7 gene
Compartment Confidence
mitochondrion 5
nucleus 4
cytosol 2

No data available for Gene Ontology (GO) - Cellular Components for COQ7 Gene

Pathways & Interactions for COQ7 Gene

genes like me logo Genes that share pathways with COQ7: view

Pathways by source for COQ7 Gene

UniProtKB/Swiss-Prot Q99807-COQ7_HUMAN

  • Pathway: Cofactor biosynthesis; ubiquinone biosynthesis.

Gene Ontology (GO) - Biological Process for COQ7 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006744 ubiquinone biosynthetic process IEA --
GO:0042493 response to drug IEA --
GO:0044281 small molecule metabolic process TAS --
GO:0045944 positive regulation of transcription from RNA polymerase II promoter IMP 25961505
GO:0055114 oxidation-reduction process IEA --
genes like me logo Genes that share ontologies with COQ7: view

No data available for SIGNOR curated interactions for COQ7 Gene

Drugs & Compounds for COQ7 Gene

(1) Drugs for COQ7 Gene - From: Novoseek

Name Status Disease Links Group Role Mechanism of Action Clinical Trials

(11) Additional Compounds for COQ7 Gene - From: HMDB and Novoseek

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
2-Hexaprenyl-3-methyl-6-methoxy-1,4 benzoquinone
  • Armco iron
  • Carbonyl iron
  • FE
  • Ferrovac e
  • Hematite
genes like me logo Genes that share compounds with COQ7: view

Transcripts for COQ7 Gene

Unigene Clusters for COQ7 Gene

Coenzyme Q7 homolog, ubiquinone (yeast):
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for COQ7 Gene

ExUns: 1a · 1b · 1c ^ 2a · 2b · 2c ^ 3 ^ 4a · 4b · 4c ^ 5a · 5b · 5c ^ 6a · 6b ^ 7 ^ 8a · 8b · 8c · 8d ^ 9
SP1: - - - - - - - - - -
SP2: - - - - - - -
SP3: - - - - -
SP4: - - - - - - - - -
SP5: - - - - - -
SP6: - - - - - -
SP7: - - -
SP8: - -
SP9: - - - - -

Relevant External Links for COQ7 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for COQ7 Gene

mRNA expression in normal human tissues for COQ7 Gene

Protein differential expression in normal tissues from HIPED for COQ7 Gene

This gene is overexpressed in Tonsil (11.3), Heart (6.6), and Bone (6.6).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, MOPED, and MaxQB for COQ7 Gene

SOURCE GeneReport for Unigene cluster for COQ7 Gene Hs.157113

mRNA Expression by UniProt/SwissProt for COQ7 Gene

Tissue specificity: Expressed dominantly in heart and skeletal muscle.
genes like me logo Genes that share expression patterns with COQ7: view

Protein tissue co-expression partners for COQ7 Gene

- Elite partner

Primer Products

No data available for mRNA expression in embryonic tissues and stem cells from LifeMap Discovery and mRNA differential expression in normal tissues for COQ7 Gene

Orthologs for COQ7 Gene

This gene was present in the common ancestor of animals and fungi.

Orthologs for COQ7 Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia COQ7 35
  • 88.94 (n)
  • 89.4 (a)
COQ7 36
  • 89 (a)
(Canis familiaris)
Mammalia COQ7 35
  • 87.71 (n)
  • 85.71 (a)
COQ7 36
  • 86 (a)
(Mus musculus)
Mammalia Coq7 35
  • 81.41 (n)
  • 85.25 (a)
Coq7 16
Coq7 36
  • 85 (a)
(Pan troglodytes)
Mammalia COQ7 35
  • 98.77 (n)
  • 98.16 (a)
COQ7 36
  • 98 (a)
(Rattus norvegicus)
Mammalia Coq7 35
  • 81.41 (n)
  • 82.95 (a)
(Monodelphis domestica)
Mammalia COQ7 36
  • 74 (a)
(Ornithorhynchus anatinus)
Mammalia COQ7 36
  • 74 (a)
(Gallus gallus)
Aves COQ7 35
  • 68.39 (n)
  • 73.24 (a)
COQ7 36
  • 70 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia coq7 35
  • 65.38 (n)
  • 69.71 (a)
Str.14928 35
(Danio rerio)
Actinopterygii coq7 35
  • 64.96 (n)
  • 72.16 (a)
Dr.19634 35
coq7 36
  • 61 (a)
fruit fly
(Drosophila melanogaster)
Insecta COQ7 37
  • 57 (a)
COQ7 35
  • 59.02 (n)
  • 58.24 (a)
COQ7 36
  • 51 (a)
(Caenorhabditis elegans)
Secernentea clk-1 37
  • 56 (a)
clk-1 35
  • 58.53 (n)
  • 56.4 (a)
clk-1 36
  • 53 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_ACL162C 35
  • 52.22 (n)
  • 47.22 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0E02421g 35
  • 53.41 (n)
  • 52.05 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes CAT5 35
  • 53.93 (n)
  • 48.31 (a)
CAT5 36
  • 39 (a)
CAT5 38
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.7697 35
bread mold
(Neurospora crassa)
Ascomycetes NCU03957 35
  • 52.73 (n)
  • 47.62 (a)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes coq7 35
  • 54.35 (n)
  • 47.29 (a)
sea squirt
(Ciona savignyi)
Ascidiacea CSA.2575 36
  • 59 (a)
Species with no ortholog for COQ7:
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • African malaria mosquito (Anopheles gambiae)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • lizard (Anolis carolinensis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for COQ7 Gene

Gene Tree for COQ7 (if available)
Gene Tree for COQ7 (if available)

Paralogs for COQ7 Gene

No data available for Paralogs for COQ7 Gene

Variants for COQ7 Gene

Sequence variations from dbSNP and Humsavar for COQ7 Gene

SNP ID Clin Chr 16 pos Sequence Context AA Info Type
rs11074359 - 19,073,976(+) GGTTA(A/C/T)GTTCA nc-transcript-variant, reference, missense
rs8062203 -- 19,079,885(+) AGTCT(A/G)CGAAT nc-transcript-variant, utr-variant-3-prime
rs8062491 -- 19,079,799(+) GTAGT(A/G)GACAC nc-transcript-variant, utr-variant-3-prime
rs4346187 -- 19,069,852(+) GATCT(C/T)AGCTC intron-variant
rs12935719 -- 19,071,428(+) accgc(G/T)cccgg intron-variant

Structural Variations from Database of Genomic Variants (DGV) for COQ7 Gene

Variant ID Type Subtype PubMed ID
esv587 CNV CNV 17122850
dgv823e1 CNV Complex 17122850
esv34419 CNV Gain 17911159
nsv436835 CNV Insertion 17901297
dgv2640n71 CNV Gain 21882294
nsv103187 CNV Loss 16902084

Variation tolerance for COQ7 Gene

Residual Variation Intolerance Score: 85.1% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 2.64; 45.51% of all genes are more intolerant (likely to be disease-causing)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot and Relevant External Links for COQ7 Gene

Disorders for COQ7 Gene

MalaCards: The human disease database

(1) MalaCards diseases for COQ7 Gene - From: ClinVar and GeneTests

Disorder Aliases PubMed IDs
coq7-related coenzyme q10 deficiency
  • coenzyme q10 deficiency, primary, 8
- elite association - COSMIC cancer census association via MalaCards
Search COQ7 in MalaCards View complete list of genes associated with diseases

Relevant External Links for COQ7

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
Atlas of Genetics and Cytogenetics in Oncology and Haematology:
genes like me logo Genes that share disorders with COQ7: view

No data available for UniProtKB/Swiss-Prot and Genatlas for COQ7 Gene

Publications for COQ7 Gene

  1. Structural and functional conservation of the Caenorhabditis elegans timing gene clk-1. (PMID: 9020081) Ewbank J.J. … Hekimi S. (Science 1997) 2 3 4 67
  2. The anti-neurodegeneration drug clioquinol inhibits the aging-associated protein CLK-1. (PMID: 18927074) Wang Y. … Hekimi S. (J. Biol. Chem. 2009) 3 23
  3. Identification and chromosomal location of two human genes encoding enzymes potentially involved in proteolytic maturation of farnesylated proteins. (PMID: 10373325) Freije J.M.P. … Lopez-Otin C. (Genomics 1999) 2 3
  4. Orthologues of the Caenorhabditis elegans longevity gene clk-1 in mouse and human. (PMID: 10373327) Asaumi S. … Shirasawa T. (Genomics 1999) 3 23
  5. Conservation of the Caenorhabditis elegans timing gene clk-1 from yeast to human: a gene required for ubiquinone biosynthesis with potential implications for aging. (PMID: 10501970) Vajo Z. … Francomano C.A. (Mamm. Genome 1999) 3 23

Products for COQ7 Gene

Sources for COQ7 Gene