COQ7
COQ7 (Coenzyme Q7, Hydroxylase) is a Protein Coding gene. Diseases associated with COQ7 include Coenzyme Q10 Deficiency, Primary, 8. Among its related pathways are Ubiquinol biosynthesis and Metabolism.
Full Name
Coenzyme Q7, Hydroxylase
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.
Biological Process
Determination of adult lifespan Source: GO_Central
Negative regulation of transcription by RNA polymerase II Source: WormBase
Positive regulation of transcription by RNA polymerase II Source: WormBase
Regulation of gene expression Source: GO_Central
Regulation of reactive oxygen species metabolic process Source: WormBase
Ubiquinone biosynthetic process Source: GO_Central
Negative regulation of transcription by RNA polymerase II Source: WormBase
Positive regulation of transcription by RNA polymerase II Source: WormBase
Regulation of gene expression Source: GO_Central
Regulation of reactive oxygen species metabolic process Source: WormBase
Ubiquinone biosynthetic process Source: GO_Central
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Coenzyme Q10 deficiency, primary, 8 (COQ10D8):
An autosomal recessive disorder resulting from mitochondrial dysfunction and characterized by decreased levels of coenzyme Q10. Patients manifest neonatal lung hypoplasia, contractures, early infantile hypertension and cardiac hypertrophy, secondary to prenatal kidney dysplasia, with neonatal and infantile renal dysfunction. Clinical features also include progressive peripheral neuropathy, muscular hypotonia and atrophy, and mild psychomotor delay with hearing and visual impairment.
An autosomal recessive disorder resulting from mitochondrial dysfunction and characterized by decreased levels of coenzyme Q10. Patients manifest neonatal lung hypoplasia, contractures, early infantile hypertension and cardiac hypertrophy, secondary to prenatal kidney dysplasia, with neonatal and infantile renal dysfunction. Clinical features also include progressive peripheral neuropathy, muscular hypotonia and atrophy, and mild psychomotor delay with hearing and visual impairment.
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Anti-COQ7 antibodies
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CompareTarget: COQ7
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 06
Application*: E
Target: COQ7
Expressed Host: HEK293 Cells
Sequence: Amino Acid: 37-217
Tag: His Tag
Target: COQ7
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human, Mouse, Rat
Clone: CBYY-C2063
Application*: WB, IP, IF, E, P
Target: COQ7
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Human, Mouse, Rat
Clone: CBFYC-2100
Application*: WB, IP, IF, E
Target: COQ7
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: 01
Application*: E
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For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
- AActivation
- AGAgonist
- APApoptosis
- BBlocking
- BABioassay
- BIBioimaging
- CImmunohistochemistry-Frozen Sections
- CIChromatin Immunoprecipitation
- CTCytotoxicity
- CSCostimulation
- DDepletion
- DBDot Blot
- EELISA
- ECELISA(Cap)
- EDELISA(Det)
- ESELISpot
- EMElectron Microscopy
- FFlow Cytometry
- FNFunction Assay
- GSGel Supershift
- IInhibition
- IAEnzyme Immunoassay
- ICImmunocytochemistry
- IDImmunodiffusion
- IEImmunoelectrophoresis
- IFImmunofluorescence
- IGImmunochromatography
- IHImmunohistochemistry
- IMImmunomicroscopy
- IOImmunoassay
- IPImmunoprecipitation
- ISIntracellular Staining for Flow Cytometry
- LALuminex Assay
- LFLateral Flow Immunoassay
- MMicroarray
- MCMass Cytometry/CyTOF
- MDMeDIP
- MSElectrophoretic Mobility Shift Assay
- NNeutralization
- PImmunohistologyp-Paraffin Sections
- PAPeptide Array
- PEPeptide ELISA
- PLProximity Ligation Assay
- RRadioimmunoassay
- SStimulation
- SESandwich ELISA
- SHIn situ hybridization
- TCTissue Culture
- WBWestern Blot
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