Connexin 31, Polyclonal Antibody

Gap junction beta-3 protein; Connexin-31; Cx31; GJB3; CX31

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This Connexin 31 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 227-257 amino acids from the C-terminal region of human Connexin 31.

Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Vial Concentration: 2 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-GJB3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Gap junctions are conduits that allow the direct cell-to-cell passage of small cytoplasmic molecules, including ions, metabolic intermediates, and second messengers, and thereby mediate intercellular metabolic and electrical communication. Gap junction channels consist of connexin protein subunits, which are encoded by a multigene family. GJBs (gap-junction proteins or connexins) play crucial functional roles associated with these channels. Defects in GJB3 have been linked to erythrokeratodermia variabilis (EKV) is an autosomal dominant genodermatosis characterized by transient figurate red patches or hyperkeratosis. Mutations in GJB2 have also been associated with genetically derived hearing impairments, including autosomal recessive nonsyndromic deafness.

Neuroscience; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)

WB~~1:1000

Western blot analysis of hGJB3-C241 in WiDr cell line lysates (35ug/lane). GJB3 (arrow) was detected using the purified Pab.(2ug/ml)

Formalin-fixed and paraffin-embedded human cancer tissue reacted with the primary antibody, which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated. BC = breast carcinoma; HC = hepatocarcinoma.

30818

gap junction protein, beta 3, 31kDa

gap junction beta-3 protein

Gap junction beta-3 protein

CX31; Cx31  [Similar Products]

CXB3_HUMAN

This gene is a member of the connexin gene family. The encoded protein is a component of gap junctions, which are composed of arrays of intercellular channels that provide a route for the diffusion of low molecular weight materials from cell to cell. Mutations in this gene can cause non-syndromic deafness or erythrokeratodermia variabilis, a skin disorder. Alternative splicing results in multiple transcript variants encoding the same protein. [provided by RefSeq, Jul 2008]

GJB3: One gap junction consists of a cluster of closely packed pairs of transmembrane channels, the connexons, through which materials of low MW diffuse from one cell to a neighboring cell. Defects in GJB3 are a cause of erythrokeratodermia variabilis (EKV). EKV is a genodermatosis characterized by the appearance of two independent skin lesions: transient figurate erythematous patches and hyperkeratosis that is usually localized but occasionally occurs in its generalized form. Clinical presentation varies significantly within a family and from one family to another. Palmoplantar keratoderma is present in around 50% of cases. Defects in GJB3 are the cause of deafness autosomal dominant type 2B (DFNA2B). DFNA2 is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. Belongs to the connexin family. Beta-type (group I) subfamily.

Protein type: Channel, misc.; Membrane protein, multi-pass; Membrane protein, integral

Chromosomal Location of Human Ortholog: 1p34

Cellular Component: connexon complex; cytoplasm; integral to membrane; gap junction

Molecular Function: gap junction channel activity

Biological Process: skin development; sensory perception of sound; in utero embryonic development; cell communication; transmembrane transport; placenta development

Disease: Deafness, Autosomal Recessive 1a; Deafness, Autosomal Dominant 2b; Erythrokeratodermia Variabilis Et Progressiva

Mhatre, A.N., et al., Clin. Genet. 63(2):154-159 (2003).
Diestel, S., et al., Biochem. Biophys. Res. Commun. 296(3):721-728 (2002).
Di, W.L., et al., Hum. Mol. Genet. 11(17):2005-2014 (2002).
Gottfried, I., et al., Hum. Mol. Genet. 11(11):1311-1316 (2002).
Wenzel, K., et al., Biochem. Biophys. Res. Commun. 248(3):910-915 (1998).

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