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Author Notes:

Correspondence: Email: sunyu@hust.edu.cn; (Y.S.); Email: entwjkong@hust.edu.cn (W.-J.K.); Tel.: +86-27-8535-1632 (Y.S.); +86-27-8535-1706 (W.-J.K.); Fax: +86-27-8577-6343 (Y.S. & W.-J.K.) These authors contributed equally to this work.

Yu Sun, Wei-Jia Kong and Xi Lin conceived and designed the experiments; Xing-Xing Zhou, Sen Chen, Le Xie, Yu-Zi Ji and Qi Yang performed the experiments; Xing-Xing Zhou, Sen Chen and Yu Sun analyzed the data; Xing-Xing Zhou, Wen-Wen Wang, Jin-Tao Yu and Xia Wu contributed the mouse breeding; Sen Chen and Yu Sun wrote the paper.

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Research Funding:

This work was supported by grants from the Key Project of National Natural Science Foundation of China (81230021), the Major State Basic Research Development Program of China (973 Program) (2011CB504504), International Science & Technology Cooperation Program of China (2013DFE33160) and the National Nature Science Foundation of China (81000408, 81570923, 81470696, 81500793 and 81500795).

Keywords:

  • Connexin26
  • GJB2
  • noise-induced hearing loss

Reduced connexin26 in the mature cochlea increases susceptibility to noise-induced hearing lossin mice

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Journal Title:

International Journal of Molecular Sciences

Volume:

Volume 17, Number 3

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Article | Final Publisher PDF

Abstract:

Connexin26 (Cx26, encoded by GJB2) mutations are the most common cause of non-syndromic deafness. GJB2 is thought to be involved in noise-induced hearing loss (NIHL). However, the role of Cx26 in NIHL is still obscure. To explore the association between Cx26 and NIHL, we established a Cx26 knockdown (KD) mouse model by conditional knockdown of Cx26 at postnatal day 18 (P18), and then we observed the auditory threshold and morphologic changes in these mice with or without noise exposure. The Cx26 KD mice did not exhibit substantial hearing loss and hair cell degeneration, while the Cx26 KD mice with acoustic trauma experienced higher hearing loss than simple noise exposure siblings and nearly had no recovery. Additionally, extensive outer hair cell loss and more severe destruction of the basal organ of Corti were observed in Cx26 KD mice after noise exposure. These data indicate that reduced Cx26 expression in the mature mouse cochlea may increase susceptibility to noise-induced hearing loss and facilitate the cell degeneration in the organ of Corti.

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© 2016 by the authors; licensee MDPI, Basel, Switzerland.

This is an Open Access work distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/).

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