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Mice Harbouring An Oculodentodigital Dysplasia-Linked Cx43 G60s Mutation Have Severe Hearing Loss, Julia M. Abitbol, John J. Kelly, Kevin J. Barr, Brian L. Allman, Dale W. Laird

Anatomy and Cell Biology Publications

Given the importance of connexin43 (Cx43, encoded by GJA1) function in the central nervous system and sensory organ processing, we proposed that it would also be crucial in auditory function. To that end, hearing was examined in two mouse models of oculodentodigital dysplasia that globally express GJA1 mutations resulting in mild or severe loss of Cx43 function. Although Cx43(I130T/+) mutant mice, with similar to 50% Cx43 channel function, did not have any hearing loss, Cx43(G60S/+) mutant mice, with similar to 20% Cx43 channel function, had severe hearing loss. There was no evidence of inner ear sensory hair cell loss, suggesting …