Mouse Anti-COQ8B Recombinant Antibody (CBYC-A942) (CBMAB-A4328-YC)

Provided herein is a Mouse monoclonal antibody against Human Coenzyme Q8B. The antibody can be used for immunoassay techniques, such as ELISA, WB.
See all COQ8B antibodies

Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

CBYC-A942

Antibody Isotype

IgG2b, κ

Application

ELISA, WB

Basic Information

Specificity

Human

Antibody Isotype

IgG2b, 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!]

Format

Liquid in PBS, pH 7.2

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 Q8B

Introduction

COQ8B (Coenzyme Q8B) is a protein coding gene. Diseases associated with COQ8B include Nephrotic Syndrome, Type 9 and Focal Segmental Glomerulosclerosis 1. And an important paralog of this gene is COQ8A.

Entrez Gene ID

79934

UniProt ID

Q96D53

Alternative Names

Coenzyme Q8B; AarF Domain-Containing Protein Kinase 4; AarF Domain Containing Kinase 4; Coenzyme Q Protein 8B; ADCK4; Atypical Kinase COQ8B, Mitochondrial;

Function

Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration (PubMed:24270420).

Its substrate specificity is unclear: does not show any protein kinase activity. Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway. Required for podocyte migration (PubMed:24270420).

Biological Process

Cerebellar Purkinje cell layer morphogenesis Source: UniProtKB
Protein phosphorylation Source: GOC
Ubiquinone biosynthetic process Source: UniProtKB

Cellular Location

Mitochondrion membrane; Cell membrane; Cytosol

Involvement in disease

Nephrotic syndrome 9 (NPHS9):
A form of nephrotic syndrome, a renal disease clinically characterized by progressive renal failure, severe proteinuria, hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show focal segmental glomerulosclerosis.

Topology

Helical: 92-108

References

Fareed, M., Makkar, V., Angral, R., Afzal, M., & Singh, G. (2021). Whole-exome sequencing reveals a novel homozygous mutation in the COQ8B gene associated with nephrotic syndrome. Scientific reports, 11(1), 1-11.

AbuMaziad, A. S., Thaker, T. M., Tomasiak, T. M., Chong, C. C., Galindo, M. K., & Hoyme, H. E. (2021). The role of novel COQ8B mutations in glomerulopathy and related kidney defects. American Journal of Medical Genetics Part A, 185(1), 60-67.

Maeoka, Y., Aizawa, M., Miyasako, K., Hirashio, S., Masuda, Y., Kishita, Y., ... & Masaki, T. (2020). A case report of adult-onset COQ8B nephropathy presenting focal segmental glomerulosclerosis with granular swollen podocytes. BMC nephrology, 21(1), 1-7.

Song, X., Fang, X., Tang, X., Cao, Q., Zhai, Y., Chen, J., ... & Rao, J. (2020). COQ8B nephropathy: Early detection and optimal treatment. Molecular genetics & genomic medicine, 8(8), e1360.

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Protocols

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Associated Products

Isotype control

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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