Introduction Gap junctions are formed by plaques of functionally assembled intercellular channels which are responsible for direct communication between the cytosols of adjacent cells in the form of ions and secondmessenger molecules. These channels are formed as two hexameric hemichannels of connexin (cnx) subunits that interlock head-on [ 1-61, Connexiris form an extensive family of well cons...

OBJECTIVES/HYPOTHESIS To examine the relationship between hearing and connexin 43, a dominant gap junctional protein in the central nervous system. STUDY DESIGN Original research. METHODS Connexin 43 heterozygous mice are used to assess its mutational effect on hearing. Results are compared to controls consisting of connexin 43, wild type and CBA/J mice. Hearing is assessed using auditory b...

Hearing loss due to mutations in the connexin gene family, which encodes gap junctional proteins, is a common form of hereditary deafness. In particular, connexin 26 (Cx26, GJB2) mutations are responsible for ~50% of non-syndromic hearing loss, which is the highest incidence of genetic disease. In the clinic, Cx26 mutations cause various auditory phenotypes ranging from profound congenital deaf...