Rabbit Anti-GJB3/FITC Conjugated antibody

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Connexin 31; Connexin-31; Connexin31; CX 31; Cx31; CXB3_HUMAN; DFNA 2; DFNA2; DFNA2B; EKV; FLJ22486; Gap junction beta 3 protein; Gap junction beta-3 protein; Gap junction protein beta 3 31kDa; Gap junction protein beta 3; GJB 3; Gjb3; MGC102938.

Cat:

SL2383R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Dog,Pig,Cow,Horse,Rabbit,Guinea Pig,)

Immunogen：

KLH conjugated synthetic peptide derived from human Connexin-31

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

31kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
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].

Function:
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.

Subunit:
A connexon is composed of a hexamer of connexins. Interacts with CNST.

Subcellular Location:
Cell membrane; Multi-pass membrane protein. Cell junction, gap junction.

DISEASE:
Defects in GJB3 are a cause of erythrokeratodermia variabilis (EKV) [MIM:13640]. 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) [MIM:612644]. 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.

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

Database links:

Entrez Gene: 2707 Human

Entrez Gene: 14620 Mouse

Entrez Gene: 29585 Rat

Omim: 603324 Human

SwissProt: O75712 Human

SwissProt: P28231 Mouse

SwissProt: P25305 Rat

Unigene: 522561 Human

Unigene: 90003 Mouse

Unigene: 162823 Rat

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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