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Critical role of spectrin in hearing development and deafness

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...

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Autores principales: Liu, Yan, Qi, Jieyu, Chen, Xin, Tang, Mingliang, Chu, Cenfeng, Zhu, Weijie, Li, Hui, Tian, Cuiping, Yang, Guang, Zhong, Chao, Zhang, Ying, Ni, Guangjian, He, Shuijin, Chai, Renjie, Zhong, Guisheng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Association for the Advancement of Science 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6469942/
https://www.ncbi.nlm.nih.gov/pubmed/31001589
http://dx.doi.org/10.1126/sciadv.aav7803
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author Liu, Yan
Qi, Jieyu
Chen, Xin
Tang, Mingliang
Chu, Cenfeng
Zhu, Weijie
Li, Hui
Tian, Cuiping
Yang, Guang
Zhong, Chao
Zhang, Ying
Ni, Guangjian
He, Shuijin
Chai, Renjie
Zhong, Guisheng
author_facet Liu, Yan
Qi, Jieyu
Chen, Xin
Tang, Mingliang
Chu, Cenfeng
Zhu, Weijie
Li, Hui
Tian, Cuiping
Yang, Guang
Zhong, Chao
Zhang, Ying
Ni, Guangjian
He, Shuijin
Chai, Renjie
Zhong, Guisheng
author_sort Liu, Yan
collection PubMed
description 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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spelling pubmed-64699422019-04-18 Critical role of spectrin in hearing development and deafness Liu, Yan Qi, Jieyu Chen, Xin Tang, Mingliang Chu, Cenfeng Zhu, Weijie Li, Hui Tian, Cuiping Yang, Guang Zhong, Chao Zhang, Ying Ni, Guangjian He, Shuijin Chai, Renjie Zhong, Guisheng Sci Adv Research Articles 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. American Association for the Advancement of Science 2019-04-17 /pmc/articles/PMC6469942/ /pubmed/31001589 http://dx.doi.org/10.1126/sciadv.aav7803 Text en Copyright © 2019 The Authors, some rights reserved; exclusive licensee American Association for the Advancement of Science. No claim to original U.S. Government Works. Distributed under a Creative Commons Attribution NonCommercial License 4.0 (CC BY-NC). http://creativecommons.org/licenses/by-nc/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial license (http://creativecommons.org/licenses/by-nc/4.0/) , which permits use, distribution, and reproduction in any medium, so long as the resultant use is not for commercial advantage and provided the original work is properly cited.
spellingShingle Research Articles
Liu, Yan
Qi, Jieyu
Chen, Xin
Tang, Mingliang
Chu, Cenfeng
Zhu, Weijie
Li, Hui
Tian, Cuiping
Yang, Guang
Zhong, Chao
Zhang, Ying
Ni, Guangjian
He, Shuijin
Chai, Renjie
Zhong, Guisheng
Critical role of spectrin in hearing development and deafness
title Critical role of spectrin in hearing development and deafness
title_full Critical role of spectrin in hearing development and deafness
title_fullStr Critical role of spectrin in hearing development and deafness
title_full_unstemmed Critical role of spectrin in hearing development and deafness
title_short Critical role of spectrin in hearing development and deafness
title_sort critical role of spectrin in hearing development and deafness
topic Research Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6469942/
https://www.ncbi.nlm.nih.gov/pubmed/31001589
http://dx.doi.org/10.1126/sciadv.aav7803
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