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


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYC-2101
Antibody Isotype
IgG1, κ
Application
WB, IF

Basic Information

Immunogen
Recombinant human CABC1.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:100-1:1,000
IF(ICC)1:50-1:500

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
0.1% gelatin
Preservative
0.09% sodium azide
Concentration
0.1 mg/ml
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Coenzyme Q8A
Entrez Gene ID
56997
UniProt ID
Q8NI60
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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For research use only. Not intended for any clinical use.

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