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Sci Adv. 2019 Apr 17;5(4):eaav7803. doi: 10.1126/sciadv.aav7803. eCollection 2019 Apr.

Critical role of spectrin in hearing development and deafness.

Liu Y1, Qi J1,2,3, Chen X4, Tang M2,3, Chu C1,4, Zhu W1,2,3, Li H1, Tian C1, Yang G5, Zhong C6, Zhang Y7, Ni G8,9, He S4, Chai R2,3,10,11,12, Zhong G1,3,4,13.

Author information

1
iHuman Institute, ShanghaiTech University, Shanghai, China.
2
Key Laboratory for Developmental Genes and Human Disease, Ministry of Education, Institute of Life Sciences, Jiangsu Province High-Tech Key Laboratory for Bio-Medical Research, Southeast University, Nanjing, China.
3
Co-innovation Center of Neuroregeneration, Jiangsu Key Laboratory of Neuroregeneration, Nantong University, Nantong, China.
4
School of Life Science and Technology, ShanghaiTech University, Shanghai, China.
5
Shanghai Institute for Advanced Immunochemical Studies, ShanghaiTech University, Shanghai, China.
6
School of Physical Science and Technology, ShanghaiTech University, Shanghai, China.
7
Department of Medical Neuroscience, Faculty of Medicine, Dalhousie University, 5850 College Street, Halifax, Canada.
8
Academy of Medical Engineering and Translational Medicine, Tianjin University, Tianjin, China.
9
Laboratory of Neural Engineering and Rehabilitation, Department of Biomedical Engineering, College of Precision Instruments and Optoelectronics Engineering, Tianjin University, Tianjin, China.
10
Institute for Stem Cell and Regeneration, Chinese Academy of Sciences, Beijing, China.
11
Research Institute of Otolaryngology, No.321 Zhongshan Road, Nanjing, China.
12
Beijing Key Laboratory of Neural Regeneration and Repair, Capital Medical University, Beijing, 100069, China.
13
Guangzhou Institute of Biomedicine and Health, Chinese Academy of Sciences, Guangzhou, China.

Abstract

Inner ear hair cells (HCs) detect sound through the deflection of mechanosensory stereocilia. Stereocilia are inserted into the cuticular plate of HCs by parallel actin rootlets, where they convert sound-induced mechanical vibrations into electrical signals. The molecules that support these rootlets and enable them to withstand constant mechanical stresses underpin our ability to hear. However, the structures of these molecules have remained unknown. We hypothesized that αII- and βII-spectrin subunits fulfill this role, and investigated their structural organization in rodent HCs. Using super-resolution fluorescence imaging, we found that spectrin formed ring-like structures around the base of stereocilia rootlets. These spectrin rings were associated with the hearing ability of mice. Further, HC-specific, βII-spectrin knockout mice displayed profound deafness. Overall, our work has identified and characterized structures of spectrin that play a crucial role in mammalian hearing development.

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