Mice harbouring an oculodentodigital dysplasia-linked Cx43 G60S mutation have severe hearing loss.
J Cell Sci
; 131(9)2018 05 04.
Article
em En
| MEDLINE
| ID: mdl-29618634
ABSTRACT
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 Cx43I130T/+ mutant mice, with â¼50% Cx43 channel function, did not have any hearing loss, Cx43G60S/+ mutant mice, with â¼20% Cx43 channel function, had severe hearing loss. There was no evidence of inner ear sensory hair cell loss, suggesting that the mechanism for Cx43-linked hearing loss lies downstream in the auditory pathway. Since evidence suggests that Cx26 function is essential for hearing and may be protective against noise-induced hearing loss, we challenged Cx43I130T/+ mice with a loud noise and found that they had a similar susceptibility to noise-induced hearing loss to that found in controls, suggesting that decreased Cx43 function does not sensitize the mice for environmentally induced hearing loss. Taken together, this study suggests that Cx43 plays an important role in baseline hearing and is essential for auditory processing.This article has an associated First Person interview with the first author of the paper.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Anormalidades Dentárias
/
Deformidades Congênitas do Pé
/
Anormalidades do Olho
/
Conexina 43
/
Sindactilia
/
Anormalidades Craniofaciais
/
Perda Auditiva
/
Mutação
Limite:
Animals
Idioma:
En
Revista:
J Cell Sci
Ano de publicação:
2018
Tipo de documento:
Article