Rabbit Anti-COQ2 Recombinant Antibody (CBXC-0528) (CBMAB-C3138-CQ)

Name: Rabbit Anti-COQ2 Recombinant Antibody (CBXC-0528)
SKU: CBMAB-C3138-CQ
Rating: 5 (1 reviews)

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Datasheet

Summary

Host Animal

Rabbit

Specificity

Human, Mouse, Rat

Clone

CBXC-0528

Antibody Isotype

IgG

Application

WB, IF, FC

Basic Information

Immunogen

A synthesized peptide derived from CSN8 (180-209aa).

Host Species

Rabbit

Specificity

Human, Mouse, Rat

Antibody Isotype

IgG

Clonality

Monoclonal Antibody

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Application	Note
WB	1:1,000-1:5,000
IF(ICC)	1:1,000
FC	1:2,000

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

Format

Liquid

Buffer

PBS, pH 7.4, 50% glycerol

Preservative

0.02% sodium azide

Concentration

Batch dependent

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 Q2 homolog, prenyltransferase (yeast)

Introduction

COQ2 (Coenzyme Q2, Polyprenyltransferase) is a Protein Coding gene. Diseases associated with COQ2 include Coenzyme Q10 Deficiency, Primary, 1 and Multiple System Atrophy 1. Among its related pathways are Ubiquinol biosynthesis and Metabolism. Gene Ontology (GO) annotations related to this gene include prenyltransferase activity and 4-hydroxybenzoate nonaprenyltransferase activity.

Entrez Gene ID

Human	27235
Mouse	71883
Rat	498332

UniProt ID

Human	Q96H96
Mouse	Q66JT7
Rat	Q499N4

Alternative Names

Coenzyme Q2, Polyprenyltransferase; 4-Hydroxybenzoate Decaprenyltransferase; 4-HB Polyprenyltransferase; PHB:Polyprenyltransferase; PHB:PPT; CL640; Para-Hydroxybenzoate-Polyprenyltransferase, Mitochondrial; 4-Hydroxybenzoate Polyprenyltransferase, Mitochondrial; Coenzyme Q2 4-Hydroxybenzoate Polyprenyltransferase; Coenzyme Q2 Homolog, Prenyltransferase (Yeast);

Function

Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl group. Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis, which is the condensation of the polyisoprenoid side chain with PHB, generating the first membrane-bound Q intermediate.

Biological Process

Glycerol metabolic process Source: UniProtKB
Isoprenoid biosynthetic process Source: UniProtKB-UniRule
Ubiquinone biosynthetic process Source: UniProtKB

Cellular Location

Mitochondrion inner membrane

Involvement in disease

Coenzyme Q10 deficiency, primary, 1 (COQ10D1):
An autosomal recessive disorder with variable manifestations consistent with 5 major phenotypes. The phenotypes include an encephalomyopathic form with seizures and ataxia; a multisystem infantile form with encephalopathy, cardiomyopathy and renal failure; a predominantly cerebellar form with ataxia and cerebellar atrophy; Leigh syndrome with growth retardation; and an isolated myopathic form.
Multiple system atrophy 1 (MSA1):
A progressive neurodegenerative disorder clinically characterized by parkinsonism, cerebellar ataxia, and autonomic, urogenital, and pyramidal dysfunction in various combinations. Pathologically, it is characterized by degeneration of striatonigral and olivopontocerebellar structures, and glial cytoplasmic inclusions that consist of abnormally phosphorylated alpha-synuclein or tau.

Topology

Mitochondrial matrix: 35-83
Helical: 84-104
Mitochondrial intermembrane: 105-108
Helical: 109-129
Mitochondrial matrix: 130-148
Helical: 149-169
Mitochondrial intermembrane: 170-172
Helical: 173-193
Mitochondrial matrix: 194-203
Helical: 204-224
Mitochondrial intermembrane: 225-231
Helical: 232-252
Mitochondrial matrix: 253-277
Helical: 278-298
Mitochondrial intermembrane: 299-300
Helical: 301-321
Mitochondrial matrix: 322-332
Helical: 333-353
Mitochondrial intermembrane: 354-371

More Infomation

References

Ni, H. F., Yang, Y., Li, C. Q., Zhou, T. Z., Liu, B. C., & Wang, B. (2021). Myeloid bodies caused by COQ2 mutation: a case with the coincidence of COQ2 nephropathy and IgA nephropathy. Clinical Kidney Journal.

Hsiao, J. H. T., Purushothuman, S., Jensen, P. H., Halliday, G. M., & Kim, W. S. (2019). Reductions in COQ2 expression relate to reduced ATP levels in multiple system Atrophy brain. Frontiers in Neuroscience, 1187.

Mikasa, M., Kanai, K., Li, Y., Yoshino, H., Mogushi, K., Hayashida, A., ... & Hattori, N. (2018). COQ2 variants in Parkinson’s disease and multiple system atrophy. Journal of Neural Transmission, 125(6), 937-944.

Hubacek, J. A., Adamkova, V., Zlatohlavek, L., Steiner-Mrazova, L., & Vrablik, M. (2017). COQ2 polymorphisms are not associated with increased risk of statin-induced myalgia/myopathy in the Czech population. Drug metabolism and personalized therapy, 32(4), 177-182.

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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)
Proteogenomics
Other Protocols

Associated Products

Related Products

Mouse Anti-COQ2 Recombinant Antibody (C4690)

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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Documents

Datasheet
SDS
Request for COA

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Second antibody and isotype control

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