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

This product is a mouse antibody that recognizes COQ2. The antibody CBXC-0528 can be used for immunoassay techniques such as: ELISA, WB.
See all COQ2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXC-0528
Antibody Isotype
IgG1, κ
Application
ELISA, WB

Basic Information

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!]

Format
Liquid
Buffer
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 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
27235
UniProt ID
Q96H96
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
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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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