Connexin 26/GJB2 Products

Connexin-26 / GJB2 is a gap junction consists of a cluster of closely packed pairs of transmembrane channels, the connexins, through which materials of low molecular weight diffuse from one cell to a neighboring cell. Each connexin is composed of a hexamer of connexin proteins. Connexins are a multi-gene family of highly related proteins. At least a dozen distinct connexin genes have been identified and many are expressed in a tissue-specific manner. Two distinct lineages, class I (beta) and class II (alpha), have been identified in mammals. Connexin-26 belongs to the class 1 (beta) group of connexins. Mutations in Connexin-26 are associated with genetically derived hearing impairments, including autosomal recessive non syndromic deafness.