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Aliases for COQ4 Gene

Aliases for COQ4 Gene

  • Coenzyme Q4 2 3
  • Coenzyme Q Biosynthesis Protein 4 Homolog 3 4
  • Ubiquinone Biosynthesis Protein COQ4 Homolog, Mitochondrial 3
  • Coenzyme Q4 Homolog (S. Cerevisiae) 2
  • Coenzyme Q4 Homolog (Yeast) 2
  • Coenzyme Q4 Homolog 3
  • COQ10D7 3
  • CGI-92 3

External Ids for COQ4 Gene

Summaries for COQ4 Gene

Entrez Gene Summary for COQ4 Gene

  • This gene encodes a component of the coenzyme Q biosynthesis pathway. Coenzyme Q, an essential component of the electron transport chain, shuttles electrons between complexes I or II to complex III of the mitochondrial transport chain. This protein appears to play a structural role in stabilizing a complex that contains most of the coenzyme Q biosynthesis enzymes. Mutations in this gene are associated with mitochondrial disorders linked to coenzyme Q deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2015]

GeneCards Summary for COQ4 Gene

COQ4 (Coenzyme Q4) is a Protein Coding gene.

UniProtKB/Swiss-Prot for COQ4 Gene

  • Component of the coenzyme Q biosynthetic pathway. May play a role in organizing a multi-subunit COQ enzyme complex required for coenzyme Q biosynthesis. Required for steady-state levels of other COQ polypeptides.

Gene Wiki entry for COQ4 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for COQ4 Gene

Genomics for COQ4 Gene

Genomic Location for COQ4 Gene

128,322,512 bp from pter
128,334,072 bp from pter
11,561 bases
Plus strand

Genomic View for COQ4 Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for COQ4 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for COQ4 Gene

Regulatory Elements for COQ4 Gene

Proteins for COQ4 Gene

  • Protein details for COQ4 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • A8WBK8
    • B2R958
    • Q5T4B8
    • Q96EW4

    Protein attributes for COQ4 Gene

    265 amino acids
    Molecular mass:
    29657 Da
    Quaternary structure:
    • Component of a multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9.

    Alternative splice isoforms for COQ4 Gene


neXtProt entry for COQ4 Gene

Proteomics data for COQ4 Gene at MOPED

Post-translational modifications for COQ4 Gene

  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for COQ4 Gene

No data available for DME Specific Peptides for COQ4 Gene

Domains for COQ4 Gene

Protein Domains for COQ4 Gene



  • Belongs to the COQ4 family.:
    • Q9Y3A0
genes like me logo Genes that share domains with COQ4: view

No data available for Gene Families for COQ4 Gene

Function for COQ4 Gene

Molecular function for COQ4 Gene

UniProtKB/Swiss-Prot Function: Component of the coenzyme Q biosynthetic pathway. May play a role in organizing a multi-subunit COQ enzyme complex required for coenzyme Q biosynthesis. Required for steady-state levels of other COQ polypeptides.

Phenotypes for COQ4 Gene

MGI mutant phenotypes for COQ4:
inferred from 1 alleles
GenomeRNAi human phenotypes for COQ4:
genes like me logo Genes that share phenotypes with COQ4: view

miRNA for COQ4 Gene

miRTarBase miRNAs that target COQ4

No data available for Enzyme Numbers (IUBMB) , Gene Ontology (GO) - Molecular Function , Animal Models , Transcription Factor Targeting and HOMER Transcription for COQ4 Gene

Localization for COQ4 Gene

Subcellular locations from UniProtKB/Swiss-Prot for COQ4 Gene

Isoform 1: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Subcellular locations from

Jensen Localization Image for COQ4 Gene COMPARTMENTS Subcellular localization image for COQ4 gene
Compartment Confidence
mitochondrion 5
cytosol 1
nucleus 1

Gene Ontology (GO) - Cellular Components for COQ4 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IDA --
GO:0005743 mitochondrial inner membrane IEA --
genes like me logo Genes that share ontologies with COQ4: view

Pathways for COQ4 Gene

SuperPathways for COQ4 Gene

No Data Available

UniProtKB/Swiss-Prot Q9Y3A0-COQ4_HUMAN

  • Pathway: Cofactor biosynthesis; ubiquinone biosynthesis.

Gene Ontology (GO) - Biological Process for COQ4 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006744 ubiquinone biosynthetic process IEA --
genes like me logo Genes that share ontologies with COQ4: view

No data available for Pathways by source for COQ4 Gene

Transcripts for COQ4 Gene

Unigene Clusters for COQ4 Gene

Coenzyme Q4 homolog (S. cerevisiae):
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for COQ4 Gene

ExUns: 1a · 1b · 1c · 1d ^ 2a · 2b · 2c ^ 3 ^ 4a · 4b ^ 5a · 5b · 5c ^ 6a · 6b · 6c · 6d ^ 7 ^ 8a · 8b ^ 9a · 9b ^ 10a · 10b · 10c · 10d
SP1: - - - - - - - - -
SP2: - - - - - - - - - - -
SP3: - - - - - - - - - - - - -
SP4: - - - -
SP5: -
SP7: - - - - - -
SP8: - - - - -
SP9: - -
SP10: - - - -

Relevant External Links for COQ4 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for COQ4 Gene

mRNA expression in normal human tissues for COQ4 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, and MOPED for COQ4 Gene

SOURCE GeneReport for Unigene cluster for COQ4 Gene Hs.98541

mRNA Expression by UniProt/SwissProt for COQ4 Gene

Tissue specificity: Expressed ubiquitously, but at high levels in liver, lung and pancreas.
genes like me logo Genes that share expressions with COQ4: view

Orthologs for COQ4 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for COQ4 Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia COQ4 36
  • 99.25 (n)
  • 98.87 (a)
COQ4 37
  • 98 (a)
(Bos Taurus)
Mammalia COQ4 36
  • 80.78 (n)
  • 75.18 (a)
COQ4 37
  • 85 (a)
(Canis familiaris)
Mammalia COQ4 36
  • 83.14 (n)
  • 82.51 (a)
COQ4 37
  • 81 (a)
(Mus musculus)
Mammalia Coq4 36
  • 83.33 (n)
  • 84.96 (a)
Coq4 16
Coq4 37
  • 81 (a)
(Monodelphis domestica)
Mammalia COQ4 37
  • 72 (a)
(Ornithorhynchus anatinus)
Mammalia COQ4 37
  • 73 (a)
(Rattus norvegicus)
Mammalia Coq4 36
  • 80.13 (n)
  • 80 (a)
(Gallus gallus)
Aves COQ4 36
  • 72.86 (n)
  • 73.45 (a)
COQ4 37
  • 63 (a)
(Anolis carolinensis)
Reptilia COQ4 37
  • 50 (a)
African clawed frog
(Xenopus laevis)
Amphibia Xl.15921 36
tropical clawed frog
(Silurana tropicalis)
Amphibia coq4 36
  • 68.12 (n)
  • 70.74 (a)
(Danio rerio)
Actinopterygii coq4 36
  • 61.23 (n)
  • 68.72 (a)
coq4 37
  • 56 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta COQ4_ANOGA 36
  • 51.23 (n)
  • 45.89 (a)
fruit fly
(Drosophila melanogaster)
Insecta CG32174 36
  • 55.13 (n)
  • 49.76 (a)
CG32174 37
  • 43 (a)
(Caenorhabditis elegans)
Secernentea coq-4 36
  • 48.2 (n)
  • 43.69 (a)
coq-4 37
  • 42 (a)
T03F1.2 38
  • 43 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AGL103W 36
  • 51.11 (n)
  • 40 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes COQ4 36
  • 47.23 (n)
  • 39.46 (a)
COQ4 37
  • 28 (a)
COQ4 39
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0D18865g 36
  • 44.25 (n)
  • 36.77 (a)
thale cress
(Arabidopsis thaliana)
eudicotyledons AT2G03690 36
  • 46.54 (n)
  • 41.01 (a)
(Oryza sativa)
Liliopsida Os04g0674200 36
  • 48.96 (n)
  • 38.28 (a)
bread mold
(Neurospora crassa)
Ascomycetes NCU02157 36
  • 49.7 (n)
  • 40.99 (a)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes coq4 36
  • 46.15 (n)
  • 44.34 (a)
sea squirt
(Ciona savignyi)
Ascidiacea CSA.2524 37
  • 45 (a)
Species with no ortholog for COQ4:
  • Actinobacteria (Mycobacterium tuberculosis)
  • 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)
  • 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 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)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for COQ4 Gene

Gene Tree for COQ4 (if available)
Gene Tree for COQ4 (if available)

Paralogs for COQ4 Gene

No data available for Paralogs for COQ4 Gene

Variants for COQ4 Gene

Sequence variations from dbSNP and Humsavar for COQ4 Gene

SNP ID Clin Chr 09 pos Sequence Context AA Info Type MAF
rs2240954 -- 128,333,053(-) GGAAC(A/G)GGCTG intron-variant
rs2270203 Likely benign 128,325,797(-) GATGT(C/T)GAAAT synonymous-codon, reference
rs3003600 -- 128,326,185(-) TATCT(A/G)CTAAA intron-variant
rs7022308 -- 128,325,296(+) CCTCT(C/G)TAGAA intron-variant
rs7044610 -- 128,330,707(+) tgatc(C/T)gcccg intron-variant

Structural Variations from Database of Genomic Variants (DGV) for COQ4 Gene

Variant ID Type Subtype PubMed ID
dgv8294n71 CNV Loss 21882294
nsv831728 CNV Loss 17160897

Relevant External Links for COQ4 Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

Disorders for COQ4 Gene

Relevant External Links for COQ4

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with COQ4: view

No data available for UniProtKB/Swiss-Prot for COQ4 Gene

Publications for COQ4 Gene

  1. Functional characterization of human COQ4, a gene required for coenzyme Q10 biosynthesis. (PMID: 18474229) Casarin A. … Salviati L. (Biochem. Biophys. Res. Commun. 2008) 2 3 4
  2. Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression. (PMID: 20877624) Hendrickson S.L. … O'Brien S.J. (PLoS ONE 2010) 3 49
  3. Identification of novel human genes evolutionarily conserved in Caenorhabditis elegans by comparative proteomics. (PMID: 10810093) Lai C.-H. … Lin W.-C. (Genome Res. 2000) 3 4
  4. Yeast COQ4 encodes a mitochondrial protein required for coenzyme Q synthesis. (PMID: 11469793) Belogrudov G.I. … Clarke C.F. (Arch. Biochem. Biophys. 2001) 2 3
  5. The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). (PMID: 15489334) Gerhard D.S. … Malek J. (Genome Res. 2004) 3 4

Products for COQ4 Gene

Sources for COQ4 Gene

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