Daple deficiency causes hearing loss in adult mice by inducing defects in cochlear stereocilia and apical microtubules

Daple 缺乏症通过诱导耳蜗纤毛和顶端微管缺陷导致成年小鼠听力丧失

阅读:6
作者:Yoshiyuki Ozono, Atsushi Tamura, Shogo Nakayama, Elisa Herawati, Yukiko Hanada, Kazuya Ohata, Maki Takagishi #, Masahide Takahashi #, Takao Imai #, Yumi Ohta #, Kazuo Oshima #, Takashi Sato #, Hidenori Inohara #, Sachiko Tsukita

Abstract

The V-shaped arrangement of hair bundles on cochlear hair cells is critical for auditory sensing. However, regulation of hair bundle arrangements has not been fully understood. Recently, defects in hair bundle arrangement were reported in postnatal Dishevelled-associating protein (ccdc88c, alias Daple)-deficient mice. In the present study, we found that adult Daple-/- mice exhibited hearing disturbances over a broad frequency range through auditory brainstem response testing. Consistently, distorted patterns of hair bundles were detected in almost all regions, more typically in the basal region of the cochlear duct. In adult Daple-/- mice, apical microtubules were irregularly aggregated, and the number of microtubules attached to plasma membranes was decreased. Similar phenotypes were manifested upon nocodazole treatment in a wild type cochlea culture without affecting the microtubule structure of the kinocilium. These results indicate critical role of Daple in hair bundle arrangement through the orchestration of apical microtubule distribution, and thereby in hearing, especially at high frequencies.

特别声明

1、本页面内容包含部分的内容是基于公开信息的合理引用;引用内容仅为补充信息,不代表本站立场。

2、若认为本页面引用内容涉及侵权,请及时与本站联系,我们将第一时间处理。

3、其他媒体/个人如需使用本页面原创内容,需注明“来源:[生知库]”并获得授权;使用引用内容的,需自行联系原作者获得许可。

4、投稿及合作请联系:info@biocloudy.com。