Rabbit anti-Human GJB3 Polyclonal Antibody | anti-GJB3 antibody
GJB3 (Gap Junction Beta 3 Protein, EKV, Connexin-31, CX31, DFNA2, DFNA2B) (Biotin)
WB: 1:500-1:2000
Applications are based on unconjugated antibody.
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
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: Membrane protein, integral; Membrane protein, multi-pass; Channel, misc.
Chromosomal Location of Human Ortholog: 1p34
Cellular Component: connexon complex; cytoplasm; gap junction; integral to membrane
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
Research Articles on GJB3
Similar Products
Product Notes
The GJB3 gjb3 (Catalog #AAA6418523) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The GJB3 (Gap Junction Beta 3 Protein, EKV, Connexin-31, CX31, DFNA2, DFNA2B) (Biotin) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's GJB3 can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC), Western Blot (WB). IHC: 1:50-1:100 WB: 1:500-1:2000 Applications are based on unconjugated antibody. Researchers should empirically determine the suitability of the GJB3 gjb3 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "GJB3, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
If you are ready to order, navigate to Shopping Cart and get ready to checkout.