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Aliases for COQ3 Gene

Aliases for COQ3 Gene

  • Coenzyme Q3, Methyltransferase 2 3 5
  • Polyprenyldihydroxybenzoate Methyltransferase 2 3 4
  • 3-Demethylubiquinol 3-O-Methyltransferase 3 4
  • Hexaprenyldihydroxybenzoate Methyltransferase, Mitochondrial 3
  • Ubiquinone Biosynthesis O-Methyltransferase, Mitochondrial 3
  • Coenzyme Q3 Homolog, Methyltransferase (S. Cerevisiae) 2
  • 3,4-Dihydroxy-5-Hexaprenylbenzoate Methyltransferase 3
  • Coenzyme Q3 Homolog, Methyltransferase (Yeast) 2
  • Dihydroxyhexaprenylbenzoate Methyltransferase 3
  • 3-Demethylubiquinone-10 3-Methyltransferase 3
  • 2-Polyprenyl-6-Hydroxyphenol Methylase 3
  • 2-Polyprenyl-6-Hydroxyphenyl Methylase 3
  • Coenzyme Q3 Homolog, Methyltransferase 3
  • DHHB Methyltransferase 3
  • Methyltransferase COQ3 3
  • EC 4
  • EC 4
  • DHHB-MTase 3
  • UG0215E05 3
  • BA9819.1 3
  • DHHB-MT 3
  • DHHBMT 3

External Ids for COQ3 Gene

Previous GeneCards Identifiers for COQ3 Gene

  • GC06M099685
  • GC06M099833
  • GC06M099862
  • GC06M099923
  • GC06M099817
  • GC06M097257

Summaries for COQ3 Gene

Entrez Gene Summary for COQ3 Gene

  • Ubiquinone, also known as coenzyme Q, or Q, is a critical component of the electron transport pathways of both eukaryotes and prokaryotes (Jonassen and Clarke, 2000 [PubMed 10777520]). This lipid consists of a hydrophobic isoprenoid tail and a quinone head group. The tail varies in length depending on the organism, but its purpose is to anchor coenzyme Q to the membrane. The quinone head group is responsible for the activity of coenzyme Q in the respiratory chain. The S. cerevisiae COQ3 gene encodes an O-methyltransferase required for 2 steps in the biosynthetic pathway of coenzyme Q. This enzyme methylates an early coenzyme Q intermediate, 3,4-dihydroxy-5-polyprenylbenzoic acid, as well as the final intermediate in the pathway, converting demethyl-ubiquinone to coenzyme Q. The COQ3 gene product is also capable of methylating the distinct prokaryotic early intermediate 2-hydroxy-6-polyprenyl phenol.[supplied by OMIM, Mar 2008]

GeneCards Summary for COQ3 Gene

COQ3 (Coenzyme Q3, Methyltransferase) is a Protein Coding gene. Among its related pathways are Metabolism and Ubiquinol biosynthesis. Gene Ontology (GO) annotations related to this gene include methyltransferase activity and S-adenosylmethionine-dependent methyltransferase activity.

UniProtKB/Swiss-Prot for COQ3 Gene

  • O-methyltransferase that catalyzes the 2 O-methylation steps in the ubiquinone biosynthetic pathway.

Additional gene information for COQ3 Gene

No data available for CIViC summary , Tocris Summary , Gene Wiki entry , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for COQ3 Gene

Genomics for COQ3 Gene

GeneHancer (GH) Regulatory Elements for COQ3 Gene

Promoters and enhancers for COQ3 Gene
GeneHancer (GH) Identifier GH Type GH
GH Sources Gene Association Score Total Score TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor
Binding Sites
Gene Targets
GH06J099393 Promoter/Enhancer 2 EPDnew Ensembl ENCODE 650.7 +0.2 244 1.6 SMAD1 ARNT SIN3A DMAP1 ZNF2 YY1 POLR2B ZNF766 ZNF213 E2F8 COQ3 LOC101927365 RPS3P5 FBXL4 TSTD3 PNISR FAXC
GH06J099417 Enhancer 0.6 ENCODE 12.2 -23.2 -23213 0.7 BACH1 JUNB DPF2 BATF PRDM6 IKZF1 MLLT1 ATF7 IKZF2 COQ3 LOC101927365 PNISR GC06M099409
GH06J099329 Enhancer 0.6 FANTOM5 11.4 +64.2 64223 0.3 CTCF RXRA TRIM22 REST ATF2 RAD21 ZNF217 NR2C1 ZNF143 KDM1A COQ3 FAXC POU3F2 GC06P099185
- Elite GeneHancer and/or Elite GeneHancer-gene association Download GeneHancer data from 2017 publication | Request up-to-date GeneHancer data (full dataset)

GeneHancers around COQ3 on UCSC Golden Path with GeneCards custom track

Top Transcription factor binding sites by QIAGEN in the COQ3 gene promoter:
  • E2F
  • E2F-4
  • E2F-3a
  • E2F-2
  • E2F-1
  • E2F-5
  • CBF-C
  • CBF-B
  • CBF-A
  • CBF(2)

Genomic Locations for COQ3 Gene

Genomic Locations for COQ3 Gene
24,819 bases
Minus strand
24,807 bases
Minus strand

Genomic View for COQ3 Gene

Genes around COQ3 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
COQ3 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for COQ3 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for COQ3 Gene

Proteins for COQ3 Gene

  • Protein details for COQ3 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Ubiquinone biosynthesis O-methyltransferase, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • B3KPX0
    • Q5T061
    • Q6P4F0
    • Q8IXG6
    • Q96BG1
    • Q9H0N1

    Protein attributes for COQ3 Gene

    369 amino acids
    Molecular mass:
    41054 Da
    Quaternary structure:
    • Component of a multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9.
    • Sequence=AAF66826.1; Type=Frameshift; Positions=16, 18, 22, 26, 44, 49, 60, 363, 364; Evidence={ECO:0000305};

neXtProt entry for COQ3 Gene

Post-translational modifications for COQ3 Gene

  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for COQ3 Gene

No data available for DME Specific Peptides for COQ3 Gene

Domains & Families for COQ3 Gene

Gene Families for COQ3 Gene

Protein Domains for COQ3 Gene

Suggested Antigen Peptide Sequences for COQ3 Gene

GenScript: Design optimal peptide antigens:

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the class I-like SAM-binding methyltransferase superfamily. UbiG/COQ3 family.
  • Belongs to the class I-like SAM-binding methyltransferase superfamily. UbiG/COQ3 family.
genes like me logo Genes that share domains with COQ3: view

Function for COQ3 Gene

Molecular function for COQ3 Gene

UniProtKB/Swiss-Prot Function:
O-methyltransferase that catalyzes the 2 O-methylation steps in the ubiquinone biosynthetic pathway.
UniProtKB/Swiss-Prot CatalyticActivity:
S-adenosyl-L-methionine + 3,4-dihydroxy-5-all-trans-polyprenylbenzoate = S-adenosyl-L-homocysteine + 3-methoxy-4-hydroxy-5-all-trans-polyprenylbenzoate.
UniProtKB/Swiss-Prot CatalyticActivity:
S-adenosyl-L-methionine + 3-demethylubiquinone-n = S-adenosyl-L-homocysteine + ubiquinone-n.

Enzyme Numbers (IUBMB) for COQ3 Gene

Gene Ontology (GO) - Molecular Function for COQ3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004395 hexaprenyldihydroxybenzoate methyltransferase activity IEA --
GO:0008168 methyltransferase activity IEA --
GO:0008171 O-methyltransferase activity IGI 10777520
GO:0008425 2-polyprenyl-6-methoxy-1,4-benzoquinone methyltransferase activity IEA --
GO:0008689 3-demethylubiquinone-9 3-O-methyltransferase activity IEA --
genes like me logo Genes that share ontologies with COQ3: view
genes like me logo Genes that share phenotypes with COQ3: view

Animal Model Products

  • Taconic Biosciences Mouse Models for COQ3

CRISPR Products

miRNA for COQ3 Gene

miRTarBase miRNAs that target COQ3

Inhibitory RNA Products

  • Search GeneCopoeia for shRNA, lentivirus and/or AAV clone products for COQ3

No data available for Phenotypes From GWAS Catalog , Human Phenotype Ontology , Animal Models , Transcription Factor Targets and HOMER Transcription for COQ3 Gene

Localization for COQ3 Gene

Subcellular locations from UniProtKB/Swiss-Prot for COQ3 Gene

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Subcellular locations from

Extracellular space Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi Apparatus Nucleus Mitochondrion 0 1 2 3 4 5 Confidence
COMPARTMENTS Subcellular localization image for COQ3 gene
Compartment Confidence
mitochondrion 5
peroxisome 2
nucleus 2
cytosol 1

Subcellular locations from the

Human Protein Atlas (HPA)
  • Mitochondria (2)
  • Nucleoplasm (2)
See all subcellular structures

Gene Ontology (GO) - Cellular Components for COQ3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IEA,IDA 10777520
GO:0005743 mitochondrial inner membrane IEA --
GO:0005759 mitochondrial matrix TAS --
GO:0016020 membrane IEA --
genes like me logo Genes that share ontologies with COQ3: view

Pathways & Interactions for COQ3 Gene

genes like me logo Genes that share pathways with COQ3: view

Pathways by source for COQ3 Gene

UniProtKB/Swiss-Prot Q9NZJ6-COQ3_HUMAN

  • Pathway: Cofactor biosynthesis; ubiquinone biosynthesis.

Gene Ontology (GO) - Biological Process for COQ3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006071 glycerol metabolic process IGI 10777520
GO:0006744 ubiquinone biosynthetic process TAS,IEA --
GO:0010795 regulation of ubiquinone biosynthetic process IEA --
GO:0032259 methylation IEA --
genes like me logo Genes that share ontologies with COQ3: view

No data available for SIGNOR curated interactions for COQ3 Gene

Drugs & Compounds for COQ3 Gene

(3) Drugs for COQ3 Gene - From: HMDB

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
s-adenosylhomocysteine Experimental Pharma 0
Ubiquinone-1 Experimental Pharma 0
Ubiquinone-2 Experimental Pharma 0

(12) Additional Compounds for COQ3 Gene - From: HMDB

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
  • Demethyl-ubiquinol-6
3-Hexaprenyl-4,5-Dihydroxybenzoic acid
  • 3-Hexaprenyl-4,5-dihydroxybenzoate
  • 3,4-Dihydroxy-5-hexaprenylbenzoate
  • 3,4-Dihydroxy-5-hexaprenylbenzoic acid
  • Dhhpba
genes like me logo Genes that share compounds with COQ3: view

Transcripts for COQ3 Gene

Unigene Clusters for COQ3 Gene

Coenzyme Q3 homolog, methyltransferase (S. cerevisiae):
Representative Sequences:

CRISPR Products

Inhibitory RNA Products

  • Search GeneCopoeia for shRNA, lentivirus and/or AAV clone products for COQ3

Alternative Splicing Database (ASD) splice patterns (SP) for COQ3 Gene

ExUns: 1a · 1b · 1c · 1d ^ 2 ^ 3 ^ 4a · 4b ^ 5 ^ 6a · 6b ^ 7 ^ 8a · 8b
SP1: -
SP3: - -
SP4: - -
SP5: - - - - - - -

Relevant External Links for COQ3 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for COQ3 Gene

mRNA expression in normal human tissues from GTEx, Illumina, BioGPS, and CGAP SAGE for COQ3 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for COQ3 Gene

This gene is overexpressed in Heart - Left Ventricle (x4.2).

Protein differential expression in normal tissues from HIPED for COQ3 Gene

This gene is overexpressed in Heart (22.2), Breast (11.5), and Tonsil (7.6).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, MaxQB, and MOPED for COQ3 Gene

Protein tissue co-expression partners for COQ3 Gene

- Elite partner

NURSA nuclear receptor signaling pathways regulating expression of COQ3 Gene:


SOURCE GeneReport for Unigene cluster for COQ3 Gene:


Evidence on tissue expression from TISSUES for COQ3 Gene

  • Kidney(4.4)
  • Nervous system(4.4)
  • Skin(4.3)
  • Liver(4.2)
  • Muscle(4.2)
  • Heart(2.1)
  • Gall bladder(2)
  • Intestine(2)
  • Thyroid gland(2)
genes like me logo Genes that share expression patterns with COQ3: view

No data available for mRNA Expression by UniProt/SwissProt and Phenotype-based relationships between genes and organs from Gene ORGANizer for COQ3 Gene

Orthologs for COQ3 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for COQ3 Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia COQ3 34 33
  • 99.46 (n)
(Canis familiaris)
Mammalia COQ3 34 33
  • 86.21 (n)
(Bos Taurus)
Mammalia COQ3 34 33
  • 85.73 (n)
(Rattus norvegicus)
Mammalia Coq3 33
  • 83.57 (n)
(Mus musculus)
Mammalia Coq3 16 34 33
  • 78.56 (n)
(Monodelphis domestica)
Mammalia COQ3 34
  • 66 (a)
(Ornithorhynchus anatinus)
Mammalia COQ3 34
  • 52 (a)
(Gallus gallus)
Aves COQ3 34 33
  • 70.14 (n)
(Anolis carolinensis)
Reptilia COQ3 34
  • 64 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia coq3 33
  • 65.54 (n)
African clawed frog
(Xenopus laevis)
Amphibia Xl.22548 33
(Danio rerio)
Actinopterygii coq3 34 33
  • 61.15 (n)
rainbow trout
(Oncorhynchus mykiss)
Actinopterygii Omy.4935 33
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP010537 33
  • 49.87 (n)
fruit fly
(Drosophila melanogaster)
Insecta CG9249 34 35 33
  • 49.47 (n)
(Caenorhabditis elegans)
Secernentea coq-3 34 33
  • 47.68 (n)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0C03630g 33
  • 46.05 (n)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes COQ3 36 34 33
  • 45.49 (n)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_ADR115W 33
  • 44.12 (n)
thale cress
(Arabidopsis thaliana)
eudicotyledons COQ3 33
  • 51.42 (n)
(Oryza sativa)
Liliopsida Os06g0152300 33
  • 53.14 (n)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes coq3 33
  • 47.42 (n)
bread mold
(Neurospora crassa)
Ascomycetes NCU05982 33
  • 44.07 (n)
sea squirt
(Ciona savignyi)
Ascidiacea -- 34
  • 38 (a)
Species where no ortholog for COQ3 was found in the sources mined by GeneCards:
  • 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)
  • 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 COQ3 Gene

Gene Tree for COQ3 (if available)
Gene Tree for COQ3 (if available)
Evolutionary constrained regions (ECRs) for COQ3: view image

Paralogs for COQ3 Gene

No data available for Paralogs for COQ3 Gene

Variants for COQ3 Gene

Sequence variations from dbSNP and Humsavar for COQ3 Gene

SNP ID Clin Chr 06 pos Variation AA Info Type
rs1000078027 -- 99,371,394(-) C/T intron_variant
rs1000131477 -- 99,370,953(-) A/C intron_variant
rs1000228330 -- 99,389,272(-) C/T genic_upstream_transcript_variant, intron_variant
rs1000381799 -- 99,395,205(-) T/C upstream_transcript_variant
rs1000452915 -- 99,375,721(-) A/G intron_variant

Structural Variations from Database of Genomic Variants (DGV) for COQ3 Gene

Variant ID Type Subtype PubMed ID
dgv1819e212 CNV loss 25503493
esv3571319 CNV loss 25503493
esv3890914 CNV loss 25118596
nsv1029536 CNV loss 25217958
nsv1117658 CNV deletion 24896259
nsv527517 CNV loss 19592680

Variation tolerance for COQ3 Gene

Residual Variation Intolerance Score: 87.2% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 10.82; 91.72% of all genes are more intolerant (likely to be disease-causing)

Additional Variant Information for COQ3 Gene

SNPedia medical, phenotypic, and genealogical associations of SNPs for

SNP Genotyping and Copy Number Assay Products

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for COQ3 Gene

Disorders for COQ3 Gene

Additional Disease Information for COQ3

Genetic Association Database
Human Genome Epidemiology Navigator
ATLAS of Genetics and Cytogenetics in Oncology and Haematology

No disorders were found for COQ3 Gene.

No data available for MalaCards , UniProtKB/Swiss-Prot and Genatlas for COQ3 Gene

Publications for COQ3 Gene

  1. Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis. (PMID: 10777520) Jonassen T … Clarke CF (The Journal of biological chemistry 2000) 2 3 4 22 58
  2. Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression. (PMID: 20877624) Hendrickson SL … O'Brien SJ (PloS one 2010) 3 44 58
  3. An association study of 45 folate-related genes in spina bifida: Involvement of cubilin (CUBN) and tRNA aspartic acid methyltransferase 1 (TRDMT1). (PMID: 19161160) Franke B … Blom HJ (Birth defects research. Part A, Clinical and molecular teratology 2009) 3 44 58
  4. Complete sequencing and characterization of 21,243 full-length human cDNAs. (PMID: 14702039) Ota T … Sugano S (Nature genetics 2004) 3 4 58
  5. The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). (PMID: 15489334) Gerhard DS … MGC Project Team (Genome research 2004) 3 4 58

Products for COQ3 Gene

Sources for COQ3 Gene

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