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GJB3 Rabbit pAb

     
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
IHC-P, IHC-F, IF
Primary Accession O75712
Other Accession O75712
Host Rabbit
Clonality Polyclonal
Calculated MW 30818 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human Connexin-31
Epitope Specificity 51-150/270
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SIMILARITY Belongs to the connexin family. Beta-type (group I) subfamily.
SUBUNIT A connexon is composed of a hexamer of connexins. Interacts with CNST.
DISEASE Defects in GJB3 are a cause of erythrokeratodermia variabilis (EKV) [MIM:133200]. 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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions 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].
Additional Information
Gene ID 2707
Other Names CX31; DFNA2; DFNA2B; EKV; EKVP1; Cnx31; Cxnc; D4Wsu144e; Gjb-3; CXB3_HUMAN; GJB3; Connexin-31 (Cx31); CXB3_MOUSE; Cxn-31; CXB3_RAT; gap junction protein beta 3; gap junction protein, beta 3, 31kD (connexin 31); gap junction protein, beta 3, 31kDa (connexin 31); erythrokeratodermia variabilis; gap junction protein, beta 3, 31kDa; connexin 31
Dilution IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name GJB3
Synonyms CX31
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.
Cellular Location Cell membrane; Multi-pass membrane protein. Cell junction, gap junction
Research Areas
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