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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]
COQ3 (Coenzyme Q3, Methyltransferase) is a Protein Coding gene. Diseases associated with COQ3 include Coenzyme Q10 Deficiency, Primary, 4 and Multiple Acyl-Coa Dehydrogenase Deficiency. 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.
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0004395 | hexaprenyldihydroxybenzoate methyltransferase activity | IEA | -- |
GO:0005515 | protein binding | IPI | 27499296 |
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 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 | -- |
GO:0031314 | extrinsic component of mitochondrial inner membrane | IEA | -- |
SuperPathway | Contained pathways | ||
---|---|---|---|
1 | Metabolism |
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2 | Ubiquinol biosynthesis |
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0006071 | glycerol metabolic process | IGI | 10777520 |
GO:0006744 | ubiquinone biosynthetic process | IEA,TAS | -- |
GO:0010795 | regulation of ubiquinone biosynthetic process | IEA | -- |
GO:0032259 | methylation | IEA | -- |
Name | Synonyms | Role | CAS Number | PubChem IDs | PubMed IDs | |
---|---|---|---|---|---|---|
2-Hexaprenyl-3-methyl-5-hydroxy-6-methoxy-1,4-benzoquinol |
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2-Hexaprenyl-3-methyl-5-hydroxy-6-methoxy-1,4-benzoquinone |
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2-Polyprenyl-3-methyl-5-hydroxy-6-methoxy-1,4-benzoquinone |
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3-Demethylubiquinone-9 |
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3-Hexaprenyl-4,5-Dihydroxybenzoic acid |
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63975-40-6 |
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ExUns: | 1a | · | 1b | · | 1c | · | 1d | ^ | 2 | ^ | 3 | ^ | 4a | · | 4b | ^ | 5 | ^ | 6a | · | 6b | ^ | 7 | ^ | 8a | · | 8b |
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SP1: | - | ||||||||||||||||||||||||||
SP2: | |||||||||||||||||||||||||||
SP3: | - | - | |||||||||||||||||||||||||
SP4: | - | - | |||||||||||||||||||||||||
SP5: | - | - | - | - | - | - | - |
This gene was present in the common ancestor of eukaryotes.
Organism | Taxonomy | Gene | Similarity | Type | Details |
---|---|---|---|---|---|
Chimpanzee (Pan troglodytes) |
Mammalia | COQ3 30 31 |
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OneToOne | |
Dog (Canis familiaris) |
Mammalia | COQ3 30 31 |
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OneToOne | |
Cow (Bos Taurus) |
Mammalia | COQ3 30 31 |
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OneToOne | |
Rat (Rattus norvegicus) |
Mammalia | Coq3 30 |
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Mouse (Mus musculus) |
Mammalia | Coq3 30 17 31 |
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OneToOne | |
Oppossum (Monodelphis domestica) |
Mammalia | COQ3 31 |
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OneToOne | |
Platypus (Ornithorhynchus anatinus) |
Mammalia | COQ3 31 |
|
OneToOne | |
Chicken (Gallus gallus) |
Aves | COQ3 30 31 |
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OneToOne | |
Lizard (Anolis carolinensis) |
Reptilia | COQ3 31 |
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OneToOne | |
Tropical Clawed Frog (Silurana tropicalis) |
Amphibia | coq3 30 |
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African clawed frog (Xenopus laevis) |
Amphibia | Xl.22548 30 |
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Zebrafish (Danio rerio) |
Actinopterygii | coq3 30 31 |
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OneToOne | |
Rainbow Trout (Oncorhynchus mykiss) |
Actinopterygii | Omy.4935 30 |
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African malaria mosquito (Anopheles gambiae) |
Insecta | AgaP_AGAP010537 30 |
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Fruit Fly (Drosophila melanogaster) |
Insecta | CG9249 30 31 32 |
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OneToOne | |
Worm (Caenorhabditis elegans) |
Secernentea | coq-3 30 31 |
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OneToOne | |
K. Lactis Yeast (Kluyveromyces lactis) |
Saccharomycetes | KLLA0C03630g 30 |
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Baker's yeast (Saccharomyces cerevisiae) |
Saccharomycetes | COQ3 30 31 33 |
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OneToOne | |
A. gosspyii yeast (Eremothecium gossypii) |
Saccharomycetes | AGOS_ADR115W 30 |
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Thale Cress (Arabidopsis thaliana) |
eudicotyledons | COQ3 30 |
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Rice (Oryza sativa) |
Liliopsida | Os06g0152300 30 |
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Fission Yeast (Schizosaccharomyces pombe) |
Schizosaccharomycetes | coq3 30 |
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Bread mold (Neurospora crassa) |
Ascomycetes | NCU05982 30 |
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Sea Squirt (Ciona savignyi) |
Ascidiacea | -- 31 |
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OneToOne |
SNP ID | Clinical significance and condition | Chr 06 pos | Variation | AA Info | Type |
---|---|---|---|---|---|
775079 | Likely Benign: not provided | 99,376,065(-) | C/T | MISSENSE_VARIANT | |
rs11548336 | - | p.Lys134Glu | |||
rs4144164 | - | p.Tyr329His | |||
rs6925344 | - | p.Ser272Gly |
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 |
Disorder | Aliases | PubMed IDs |
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coenzyme q10 deficiency, primary, 4 |
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multiple acyl-coa dehydrogenase deficiency |
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mitochondrial complex i deficiency, nuclear type 1 |
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mitochondrial metabolism disease |
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