WBmore details Unsuitable for:
Flow Cyt,ICC,IHC-P or IP
Mouse, Rat, Human
A synthetic peptide corresponding to residues in Human GJB3.
Human testis, HeLa and SH-SY5Y lysates
This product is a recombinant rabbit monoclonal antibody.
We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated "PUR" on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
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.
Involvement in 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.
Belongs to the connexin family. Beta-type (group I) subfamily.