Essential Role of Sptan1 in Cochlear Hair Cell Morphology and Function Via Focal Adhesion Signaling.
Qingxiu YaoHui WangHengchao ChenZhuangzhuang LiYumeng JiangZhipeng LiJiping WangYazhi XingFeng LiuDongzhen YuShankai YinPublished in: Molecular neurobiology (2021)
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.
Keyphrases
- hearing loss
- induced apoptosis
- cell cycle arrest
- single cell
- cell therapy
- genome wide
- endoplasmic reticulum stress
- type diabetes
- preterm infants
- stem cells
- oxidative stress
- cell proliferation
- cell death
- skeletal muscle
- cell migration
- gene expression
- cystic fibrosis
- high glucose
- transcription factor
- metabolic syndrome
- mesenchymal stem cells
- wild type
- smoking cessation
- quantum dots
- candida albicans