Essential Role of Sptan1 in Cochlear Hair Cell Morphology and Function Via Focal Adhesion Signaling.
Essential Role of Sptan1 in Cochlear Hair Cell Morphology and Function Via Focal Adhesion Signaling.
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Sptan1 通过局灶粘附信号传导在耳蜗毛细胞形态和功能中的重要作用。
DOI：
10.1007/s12035-021-02551-2
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发表时间：
2022-01
影响因子：
5.1
通讯作者：
Yin S
中科院分区：
文献类型：
--
作者：
Yao Q;Wang H;Chen H;Li Z;Jiang Y;Li Z;Wang J;Xing Y;Liu F;Yu D;Yin S
听力损失是人类最常见的感官缺陷。听力依赖于插入毛细胞角质板(HCS)的立体纤毛，在那里它们在声音的感知和传输中发挥着重要作用。虽然许多基因与听力损失有关，但许多毛细胞基因的功能尚未阐明。在这里，我们重点研究了非红系血影蛋白αII(SPTAN1)，它存在于角质板、立体纤毛根束周围和质膜上。有趣的是，具有HC特异性Sptan1基因敲除的小鼠在出生后早期表现出快速耳聋、立纤毛和角质板的异常形成，以及耳蜗中环和顶弯的HCS丢失。此外，Sptan1缺乏导致Corti-1细胞的内耳研究所-器官伸展减少，并导致小鼠肝细胞局灶性黏附和整合素信号的异常形成。总之，我们的发现强调SPTAN1是通过调节焦点黏附信号而对HC立体纤毛的形态和听觉功能起关键作用的分子。
Hearing loss is the most common human sensory deficit. Hearing relies on stereocilia, inserted into the cuticular plate of hair cells (HCs), where they play an important role in the perception of sound and its transmission. Although numerous genes have been associated with hearing loss, the function of many hair cell genes has yet to be elucidated. Herein, we focused on nonerythroid spectrin αII (SPTAN1), abundant in the cuticular plate, surrounding the rootlets of stereocilia and along the plasma membrane. Interestingly, mice with HC-specific Sptan1 knockout exhibited rapid deafness, abnormal formation of stereocilia and cuticular plates, and loss of HCs from middle and apical turns of the cochlea during early postnatal stages. Additionally, Sptan1 deficiency led to the decreased spreading of House Ear Institute-Organ of Corti 1 cells, and induced abnormal formation of focal adhesions and integrin signaling in mouse HCs. Altogether, our findings highlight SPTAN1 as a critical molecule for HC stereocilia morphology and auditory function via regulation of focal adhesion signaling.
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影响因子：
4.5
作者：
Forge A;Taylor RR;Dawson SJ;Lovett M;Jagger DJ
通讯作者：
Jagger DJ
影响因子：
30.8
作者：
Evans, AL;Müller, U
通讯作者：
Müller, U
影响因子：
1
作者：
Akbariazar, Elinaz;Vahabi, Ali;Rad, Isa Abdi
通讯作者：
Rad, Isa Abdi
DOI：
10.1111/j.1749-6632.1999.tb08640.x
发表时间：
1999-01-01
期刊：
OTOTOXICITY: BASIC SCIENCE AND CLINICAL APPLICATIONS
影响因子：
--
作者：
Ding, DL;Wang, J;Mueller, M
通讯作者：
Mueller, M
DOI：
10.1073/pnas.1401950111
发表时间：
2014-07-08
影响因子：
11.1
作者：
Diaz-Horta, Oscar;Subasioglu-Uzak, Asli;Tekin, Mustafa
通讯作者：
Tekin, Mustafa