Critical role of spectrin in hearing development and deafness.

Authors:
Yan Liu
Yan Liu
California Institute of Technology
Pasadena | United States
Jieyu Qi
Jieyu Qi
The Key Laboratory of Developmental Genes and Human Disease
Xin Chen
Xin Chen
College of Life Sciences
Provo | United States
Cenfeng Chu
Cenfeng Chu
iHuman Institute
China
Weijie Zhu
Weijie Zhu
Chinese PLA General Hospital
Hui Li
Hui Li
Xiangya Hospital
Sumy | Ukraine
CuiPing Tian
CuiPing Tian
Institute of Neuroscience and State Key Laboratory of Neuroscience

Sci Adv 2019 Apr 17;5(4):eaav7803. Epub 2019 Apr 17.

iHuman Institute, ShanghaiTech University, Shanghai, China.

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.

Download full-text PDF

Source
http://advances.sciencemag.org/lookup/doi/10.1126/sciadv.aav
Publisher Site
http://dx.doi.org/10.1126/sciadv.aav7803DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6469942PMC
April 2019
11 Reads

Publication Analysis

Top Keywords

hearing development
8
role investigated
4
investigated structural
4
fulfill role
4
subunits fulfill
4
αii- βii-spectrin
4
βii-spectrin subunits
4
structural organization
4
hypothesized αii-
4
super-resolution fluorescence
4
fluorescence imaging
4
hcs super-resolution
4
rodent hcs
4
organization rodent
4
imaging spectrin
4
unknown hypothesized
4
withstand constant
4
constant mechanical
4
enable withstand
4
rootlets enable
4

References

(Supplied by CrossRef)

Similar Publications