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Mice harbouring an oculodentodigital dysplasia-linked Cx43 G60S mutation have severe hearing loss.
J Cell Sci. 2018 05 04; 131(9)JC

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 ∼50% Cx43 channel function, did not have any hearing loss, Cx43G60S/+ mutant mice, with ∼20% 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.

Authors+Show Affiliations

University of Western Ontario, Department of Anatomy and Cell Biology, London, Ontario, Canada, N6A 5C1.University of Western Ontario, Department of Anatomy and Cell Biology, London, Ontario, Canada, N6A 5C1.University of Western Ontario, Department of Anatomy and Cell Biology, London, Ontario, Canada, N6A 5C1.University of Western Ontario, Department of Anatomy and Cell Biology, London, Ontario, Canada, N6A 5C1.University of Western Ontario, Department of Anatomy and Cell Biology, London, Ontario, Canada, N6A 5C1 dale.laird@schulich.uwo.ca.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

29618634

Citation

Abitbol, Julia M., et al. "Mice Harbouring an Oculodentodigital Dysplasia-linked Cx43 G60S Mutation Have Severe Hearing Loss." Journal of Cell Science, vol. 131, no. 9, 2018.
Abitbol JM, Kelly JJ, Barr KJ, et al. Mice harbouring an oculodentodigital dysplasia-linked Cx43 G60S mutation have severe hearing loss. J Cell Sci. 2018;131(9).
Abitbol, J. M., Kelly, J. J., Barr, K. J., Allman, B. L., & Laird, D. W. (2018). Mice harbouring an oculodentodigital dysplasia-linked Cx43 G60S mutation have severe hearing loss. Journal of Cell Science, 131(9). https://doi.org/10.1242/jcs.214635
Abitbol JM, et al. Mice Harbouring an Oculodentodigital Dysplasia-linked Cx43 G60S Mutation Have Severe Hearing Loss. J Cell Sci. 2018 05 4;131(9) PubMed PMID: 29618634.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Mice harbouring an oculodentodigital dysplasia-linked Cx43 G60S mutation have severe hearing loss. AU - Abitbol,Julia M, AU - Kelly,John J, AU - Barr,Kevin J, AU - Allman,Brian L, AU - Laird,Dale W, Y1 - 2018/05/04/ PY - 2017/12/20/received PY - 2018/03/27/accepted PY - 2018/4/6/pubmed PY - 2019/12/4/medline PY - 2018/4/6/entrez KW - Connexin KW - Cx43 KW - Gap junction KW - Hearing loss KW - Noise exposure JF - Journal of cell science JO - J. Cell. Sci. VL - 131 IS - 9 N2 - 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 ∼50% Cx43 channel function, did not have any hearing loss, Cx43G60S/+ mutant mice, with ∼20% 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. SN - 1477-9137 UR - https://www.unboundmedicine.com/medline/citation/29618634/Mice_harbouring_an_oculodentodigital_dysplasia_linked_Cx43_G60S_mutation_have_severe_hearing_loss_ L2 - http://jcs.biologists.org/cgi/pmidlookup?view=long&pmid=29618634 DB - PRIME DP - Unbound Medicine ER -