Connexin 26, Polyclonal Antibody

Anti -Connexin 26 (CX26, Gap Junction Beta 2 Protein, CXB-2)

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

Chromosome: 13; NC_000013.10 (20761602..20767114, complement). Location: 13q11-q12

Polyclonal

IgG

Rabbit

Recognizes mouse Connexin 26. Species sequence homology: rat-100%, human- 90%, sheep-85%.

Serum
Serum

Supplied as a liquid in 0.05% sodium azide, 40% glycerol.

May be stored at 4 degree C for short-term only. For long-term storage, aliquot and store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Small volumes of anti-GJB2 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.

Intracellular communication mediated by gap junctions plays an important role in a variety of cellular processes including: homeostasis, morphogenesis, cell differentiation, and growth control. Gap junctions are transmembrane channels that serve to directly link neighboring cells by mediating the exchange of low-molecular weight metabolites, ions, and second messengers. Gap junctions are formed by the interaction of connexons or hemichannels on adjacent cells. The connexon itself is composed of a hexameric assembly of proteins referred to as connexins. Connexins are highly homologous proteins encoded by a multigene family. The connexins exhibit similar structural features which include a cytoplasmic amino terminal region, four transmembrane domains, two extracellular loops, and a carboxyl-terminal cytoplasmic tail of varying length. Comparison of the aa sequences of the various connexin family members indicate that the two areas of greatest divergence amongst the connexin family members are the intracellular loop connecting the second and third transmembrane segments and the carboxy-terminal tail. These domains are, therefore, thought to mediate connexin-type speci c properties including: phosphorylation, responses to gating stimuli, as well as assembly and membrane turnover. Modulation of gap junctional communication can be achieved by multiple mechanisms and can occur very rapidly or over a period of several hours. These mechanisms include alterations in transcription, translation, stability, postranslational processing (especially phosphorylation), gating, and insertion or removal from the plasma membrane. Interestingly, reduction or alterations in the levels or types of connexin expressed in a given cell type has been found to correlate with tumor progression and metastasis. Mouse connexin 26 is a 226aa gap junction protein with a predicted molecular weight of ~26kD. It is prominently expressed in liver.

Antibodies; Abs to Connexin, Junction Proteins

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in ELISA and Western Blot.
Dilution: Western Blot: 1:1000-1:5000
ELISA: 1:100,000 in 50-100ng C7853-15 Connexin 26, Mouse, Control Peptide/well.

26,215 Da[Similar Products]

gap junction protein, beta 2, 26kDa

gap junction beta-2 protein; connexin 26; OTTHUMP00000018093

Gap junction beta-2 protein

CXB2_HUMAN

This gene encodes a member of the gap junction protein family. The gap junctions were first characterized by electron microscopy as regionally specialized structures on plasma membranes of contacting adherent cells. These structures were shown to consist of cell-to-cell channels that facilitate the transfer of ions and small molecules between cells. The gap junction proteins, also known as connexins, purified from fractions of enriched gap junctions from different tissues differ. According to sequence similarities at the nucleotide and amino acid levels, the gap junction proteins are divided into two categories, alpha and beta. Mutations in this gene are responsible for as much as 50% of pre-lingual, recessive deafness. [provided by RefSeq]

GJB2: 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 GJB2 are the cause of deafness autosomal recessive type 1A (DFNB1A). DFNB1A 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. Defects in GJB2 are the cause of deafness autosomal dominant type 3A (DFNA3A). Defects in GJB2 are a cause of Vohwinkel syndrome (VS). VS is an autosomal dominant disease characterized by hyperkeratosis, constriction on finger and toes and congenital deafness. Defects in GJB2 are a cause of palmoplantar keratoderma with deafness (PPKDFN). PPKDFN is an autosomal dominant disorder characterized by the association of palmoplantar hyperkeratosis with progressive, bilateral, high-frequency, sensorineural deafness. Defects in GJB2 are a cause of keratitis-ichthyosis- deafness syndrome (KID syndrome); an autosomal dominant form of ectodermal dysplasia. Ectodermal dysplasias (EDs) constitute a heterogeneous group of developmental disorders affecting tissues of ectodermal origin. EDs are characterized by abnormal development of two or more ectodermal structures such as hair, teeth, nails and sweat glands, with or without any additional clinical sign. Each combination of clinical features represents a different type of ectodermal dysplasia. KID syndrome is characterized by the association of hyperkeratotic skin lesions with vascularizing keratitis and profound sensorineural hearing loss. Clinical features include deafness, ichthyosis, photobia, absent or decreased eyebrows, sparse or absent scalp hair, decreased sweating and dysplastic finger and toenails. Defects in GJB2 are the cause of Bart-Pumphrey syndrome (BPS). BPS is an autosomal dominant disorder characterized by sensorineural hearing loss, palmoplantar keratoderma, knuckle pads, and leukonychia, It shows considerable phenotypic variability. Defects in GJB2 are the cause of ichthyosis hystrix-like with deafness syndrome (HID syndrome). HID syndrome is an autosomal-dominant inherited keratinizing disorder characterized by sensorineural deafness and spiky hyperkeratosis affecting the entire skin. HID syndrome is considered to differ from the similar KID syndrome in the extent and time of occurrence of skin symptoms and the severity of the associated keratitis. Belongs to the connexin family. Beta-type (group I) subfamily.

Protein type: Membrane protein, integral; Motility/polarity/chemotaxis; Cell adhesion; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 13q11-q12

Cellular Component: connexon complex; ER-Golgi intermediate compartment; plasma membrane; integral to membrane; lateral plasma membrane

Molecular Function: gap junction channel activity

Biological Process: sensory perception of sound; cell-cell signaling; gap junction assembly; transport; male genitalia development; decidualization; response to progesterone stimulus; transmembrane transport; response to estradiol stimulus

Disease: Deafness, Autosomal Recessive 1a; Ichthyosis, Hystrix-like, With Deafness; Deafness, X-linked 2; Deafness, Congenital, With Keratopachydermia And Constrictions Of Fingers And Toes; Keratitis-ichthyosis-deafness Syndrome, Autosomal Dominant; Knuckle Pads, Leukonychia, And Sensorineural Deafness; Keratoderma, Palmoplantar, With Deafness; Deafness, Autosomal Dominant 3a

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.