Essential Role of Sptan1 in Cochlear Hair Cell Morphology and Function Via Focal Adhesion Signaling.
Mol Neurobiol
; 59(1): 386-404, 2022 Jan.
Article
en En
| MEDLINE
| ID: mdl-34708331
ABSTRACT
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.
Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Potenciales Evocados Auditivos del Tronco Encefálico
/
Proteína-Tirosina Quinasas de Adhesión Focal
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Estereocilios
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Células Ciliadas Auditivas
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Pérdida Auditiva
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Proteínas de Microfilamentos
Límite:
Animals
Idioma:
En
Revista:
Mol Neurobiol
Asunto de la revista:
BIOLOGIA MOLECULAR
/
NEUROLOGIA
Año:
2022
Tipo del documento:
Article
País de afiliación:
China