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CLC number: R764.35

On-line Access: 2019-01-22

Received: 2016-12-02

Revision Accepted: 2017-03-26

Crosschecked: 2017-07-13

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Yen-Fu Cheng


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Journal of Zhejiang University SCIENCE B 2019 Vol.20 No.2 P.146-155


Atoh1 regulation in the cochlea: more than just transcription

Author(s):  Yen-Fu Cheng

Affiliation(s):  Department of Otology and Laryngology, Harvard Medical School, Boston, MA 02115, USA; more

Corresponding email(s):   yfcheng2@vghtpe.gov.tw

Key Words:  Atoh1, Huwe1, Cochlea, Hair cells, Regeneration, Post-translational regulation

Yen-Fu Cheng. Atoh1 regulation in the cochlea: more than just transcription[J]. Journal of Zhejiang University Science B, 2019, 20(2): 146-155.

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publisher="Zhejiang University Press & Springer",

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%I Zhejiang University Press & Springer
%DOI 10.1631/jzus.B1600438

T1 - Atoh1 regulation in the cochlea: more than just transcription
A1 - Yen-Fu Cheng
J0 - Journal of Zhejiang University Science B
VL - 20
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SP - 146
EP - 155
%@ 1673-1581
Y1 - 2019
PB - Zhejiang University Press & Springer
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DOI - 10.1631/jzus.B1600438

More than 80% of all cases of deafness are related to the death or degeneration of cochlear hair cells and the associated spiral ganglion neurons, and a lack of regeneration of these cells leads to permanent hearing loss. Therefore, the regeneration of lost hair cells is an important goal for the treatment of deafness. atoh1 is a basic helix-loop-helix (bHLH) transcription factor that is critical in both the development and regeneration of cochlear hair cells. atoh1 is transcriptionally regulated by several signaling pathways, including Notch and Wnt signalings. At the post-translational level, it is regulated through the ubiquitin-proteasome pathway. In vitro and in vivo studies have revealed that manipulation of these signaling pathways not only controls development, but also leads to the regeneration of cochlear hair cells after damage. Recent progress toward understanding the signaling networks involved in hair cell development and regeneration has led to the development of new strategies to replace lost hair cells. This review focuses on our current understanding of the signaling pathways that regulate atoh1 in the cochlea.




Darkslateblue:Affiliate; Royal Blue:Author; Turquoise:Article


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