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Mouse Anti-COQ8A Recombinant Antibody (7.5.1) (CBMAB-A4325-YC)

Provided herein is a Mouse monoclonal antibody against Human Coenzyme Q8A. The antibody can be used for immunoassay techniques, such as WB, IF.
See all COQ8A antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
7.5.1
Antibody Isotype
IgG1, κ
Application
WB, IF

Basic Information

Immunogen
CABC1 recombinant protein
Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Coenzyme Q8A
Introduction
COQ8A (Coenzyme Q8A) is a protein coding gene. Diseases associated with COQ8A include Coenzyme Q10 Deficiency, Primary, 4 and Autosomal Recessive Cerebellar Ataxia. And an important paralog of this gene is COQ8B.
Entrez Gene ID
56997
UniProt ID
Q8NI60
Alternative Names
Coenzyme Q8A; AarF Domain-Containing Protein Kinase 3; Coenzyme Q Protein 8A; ADCK3; CABC1; Chaperone, ABC1 Activity Of Bc1 Complex Homolog (S. Pombe); Chaperone, ABC1 Activity Of Bc1 Complex Like (S. Pombe); Chaperone-ABC1 (Activity Of Bc1 Complex, S.Pom
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.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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