Connexin32 can restore hearing in connexin26 deficient mice

• Published in: European journal of cell biology Vol. 90; no. 10; pp. 817 - 824
• Main Authors: Degen, Joachim, Schütz, Melanie, Dicke, Nikolai, Strenzke, Nicola, Jokwitz, Melanie, Moser, Tobias, Willecke, Klaus
• Format: Journal Article
• Language: English
• Published: Germany Elsevier GmbH 01.10.2011
• Subjects: Animals; beta-Galactosidase - biosynthesis; beta-Galactosidase - genetics; Cochlea; Connexin 26; Connexin 30; Connexins - deficiency; Connexins - genetics; Connexins - metabolism; Cx26; Cx32; Deafness; Deafness - genetics; Evoked Potentials, Auditory, Brain Stem; Gap Junction beta-1 Protein; Gap junctions; Gene Dosage; Gene Expression; Genes, Reporter; Genetic Engineering; Inner ear; Membrane Glycoproteins - genetics; Membrane Glycoproteins - metabolism; Mice; Mice, Knockout; Mice, Transgenic; Otogelin-Cre; Protein Isoforms - genetics; Protein Isoforms - metabolism; Recombinant Proteins - genetics; Recombinant Proteins - metabolism; Spiral Ligament of Cochlea - metabolism; Stria Vascularis - cytology; Stria Vascularis - metabolism; Deafness; Cx32; Gap junctions; Cochlea; Cx26; Otogelin-Cre; Inner ear

Functional gap junction channels composed of certain connexin proteins are essential for the function of the cochlea. Homozygous deficiency in the Gjb2 (mice) or GJB2 (human) gene coding for connexin26 (Cx26) in the cochlea leads to hearing impairment in mice and humans, respectively. Here we have s...