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Essential Role of Sptan1 in Cochlear Hair Cell Morphology and Function Via Focal Adhesion Signaling.
- Source :
-
Molecular neurobiology [Mol Neurobiol] 2022 Jan; Vol. 59 (1), pp. 386-404. Date of Electronic Publication: 2021 Oct 27. - Publication Year :
- 2022
-
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.<br /> (© 2021. The Author(s).)
- Subjects :
- Animals
Apoptosis physiology
Caspase 3 metabolism
Cell Line
Cell Shape physiology
Hair Cells, Auditory pathology
Hearing physiology
Hearing Loss pathology
Mice
Mice, Knockout
Microfilament Proteins genetics
Evoked Potentials, Auditory, Brain Stem physiology
Focal Adhesion Protein-Tyrosine Kinases metabolism
Hair Cells, Auditory metabolism
Hearing Loss metabolism
Microfilament Proteins metabolism
Stereocilia metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 1559-1182
- Volume :
- 59
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Molecular neurobiology
- Publication Type :
- Academic Journal
- Accession number :
- 34708331
- Full Text :
- https://doi.org/10.1007/s12035-021-02551-2