Gap junction beta-4 protein; Connexin-303; Cx303; GJB4

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

Polyclonal

Rabbit Ig

Rabbit

This Connexin 30.3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 90-120 amino acids from the N-terminal region of human Connexin 30.3.

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: 1.03 (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-GJB4 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 permit direct cell-to-cell passage of small cytoplasmic molecules, including ions, metabolic intermediates, and second messengers, and thereby mediate intercellular communication. Gap junction channels consist of connexin protein subunits encoded by a multigene family. Erythrokeratodermia variabilis (EKV) is an autosomal dominant disorder of keratinization characterized by migratory erythematous lesions and fixed keratotic plaques. Mutations in the GJB3 gene have been reported in some but not all families, although it has been postulated that the absence of connexin 30.3 can be compensated by other connexins.

Neuroscience; Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

Western blot analysis of GJB4 (arrow) using rabbit polyclonal GJB4 Antibody (H105.Connexin). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the GJB4 gene.

30419

gap junction protein, beta 4, 30.3kDa

gap junction beta-4 protein

Gap junction beta-4 protein

Cx30.3??[Similar Products]

CXB4_HUMAN

This gene encodes a transmembrane connexin protein that is a component of gap junctions. Mutations in this gene have been associated with erythrokeratodermia variabilis, progressive symmetric erythrokeratoderma and hearing impairment. [provided by RefSeq, Dec 2009]

GJB4: 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 GJB4 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. Belongs to the connexin family. Beta-type (group I) subfamily.

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

Chromosomal Location of Human Ortholog: 1p34.3

Cellular Component: connexon complex; integral to membrane

Biological Process: olfactory behavior; sensory perception of smell; cell communication

Disease: Erythrokeratodermia Variabilis Et Progressiva

Ota, T., et al., Nat. Genet. 36(1):40-45 (2004).
Richard, G., et al., J. Invest. Dermatol. 120(4):601-609 (2003).
Macari, F., et al., Am. J. Hum. Genet. 67(5):1296-1301 (2000).
Lopez-Bigas, N., et al., Hum. Mutat. 19 (4), 458 (2002).

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