COQ8A

COQ8A (Coenzyme Q8A) is a Protein Coding gene. Diseases associated with COQ8A include Coenzyme Q10 Deficiency, Primary, 4 and Autosomal Recessive Cerebellar Ataxia. An important paralog of this gene is COQ8B.
Full Name
Coenzyme Q8A
Function
Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration (PubMed:25498144, PubMed:21296186, PubMed:25540914, PubMed:27499294).

Its substrate specificity is unclear: does not show any protein kinase activity (PubMed:25498144, PubMed:27499294).

Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway, as suggested by its ability to bind coenzyme Q lipid intermediates (PubMed:25498144, PubMed:27499294).

Shows an unusual selectivity for binding ADP over ATP (PubMed:25498144).
Biological Process
Phosphorylation Source: UniProtKB
Protein phosphorylation Source: GOC
Ubiquinone biosynthetic process Source: UniProtKB
Cellular Location
Mitochondrion; Membrane
Involvement in disease
Coenzyme Q10 deficiency, primary, 4 (COQ10D4):
An autosomal recessive disorder characterized by childhood-onset of cerebellar ataxia and exercise intolerance. Patient manifest gait ataxia and cerebellar atrophy with slow progression. Additional features include brisk tendon reflexes and Hoffmann sign, variable psychomotor retardation and variable seizures.
Topology
Helical: 214-230
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Anti-COQ8A antibodies

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Mouse Anti-COQ8A Recombinant Antibody (C0637) (CBMAB-C0637-LY)

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Target: COQ8A
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: C0637
Application*: IF, WB
Datasheet SDS

Mouse Anti-COQ8A Recombinant Antibody (1C12) (CBMAB-A4324-YC)

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Target: COQ8A
Host: Mouse
Antibody Isotype: IgM, κ
Specificity: Human
Clone: 1C12
Application*: E, WB
Datasheet SDS

Mouse Anti-COQ8A Recombinant Antibody (CBXC-2514) (V2LY-1206-LY799)

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Target: COQ8A
Host: Mouse
Antibody Isotype: IgM, κ
Specificity: Human, Mouse
Clone: CBXC-2514
Application*: E, WB
Datasheet SDS

Mouse Anti-COQ8A Recombinant Antibody (CBFYC-2101) (V2LY-1206-LY798)

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Target: COQ8A
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBFYC-2101
Application*: WB, IF
Datasheet SDS

Mouse Anti-COQ8A Recombinant Antibody (A4325) (V2LY-1206-LY796)

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Target: COQ8A
Host: Mouse
Antibody Isotype: IgM, κ
Specificity: Human
Clone: A4325
Application*: E, WB
Datasheet SDS
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(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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