13-8100 reacts strongly with Connexin-26, which has a predicted molecular weight of 26.5 kDa. Reactivity of this antibody on western blots has been confirmed by using extracts from mouse liver, mouse brain, and rat brain and it appears to exhibit minimal cross-reaction with Cx30 by western blotting. The degree of cross-reaction with Cx30 by IHC is uncertain, but may be influenced by fixation conditions.
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 gap-junction proteins (GJBs) or connexins. Connexins are highly homologous proteins encoded by a multigene family. The connexins exhibit similar structural features which include a cytoplasmic N-terminal region, four transmembrane domains, two extracellular loops, and a C-terminal cytoplasmic tail of varying length. Comparison of the amino acid 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 C-terminal tail. These domains are, therefore, thought to mediate connexin-type specific 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. Defects in GJB3 have been linked to erythrokeratodermia variabilis (EKV), an autosomal dominant genodermatosis characterized by transient figurate red patches or hyperkeratosis. Mutations in GJB2 have also been associated with genetically derived hearing impairments, including autosomal recessive nonsyndromic deafness.
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Protein Aliases: 26kD (connexin 26); 26kDa (connexin 26); beta 2; Connexin 26; Connexin-26; CX26; DFNA3; DFNB1; Gap junction beta-2 protein; gap junction channel protein connexin 26; gap junction membrane channel protein beta 2; gap junction protein; gap junction protein beta 2; gap junction protein, beta 2, 26kDa; GJB2; HID; KID; mutant gap junction protein beta 2; NSRD1; PPK
Gene Aliases: AI325222; Cnx26; CX26; CXN-26; DFNA3; DFNA3A; DFNB1; DFNB1A; Gjb-2; GJB2; HID; KID; NSRD1; PPK