Murine Fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell function
Cochlear hair cells convert sound-induced vibration into electrical signals. FAM65B mutations cause hearing loss by an unknown mechanism. Using biochemistry and stochastic optical reconstruction microscopy (STORM), we show here that Fam65b oligomers form a circumferential ring near the basal taper o...
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doaj-4b1bae41c89f40fabf1f2cbbbb120bba2021-05-05T00:26:02ZengeLife Sciences Publications LtdeLife2050-084X2016-06-01510.7554/eLife.14222Murine Fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell functionBo Zhao0Zizhen Wu1Ulrich Müller2https://orcid.org/0000-0003-2736-6494Department of Molecular and Cellular Neuroscience, The Scripps Research Institute, La Jolla, United States; Dorris Neuroscience Center, The Scripps Research Institute, La Jolla, United StatesDepartment of Molecular and Cellular Neuroscience, The Scripps Research Institute, La Jolla, United States; Dorris Neuroscience Center, The Scripps Research Institute, La Jolla, United StatesDepartment of Molecular and Cellular Neuroscience, The Scripps Research Institute, La Jolla, United States; Dorris Neuroscience Center, The Scripps Research Institute, La Jolla, United StatesCochlear hair cells convert sound-induced vibration into electrical signals. FAM65B mutations cause hearing loss by an unknown mechanism. Using biochemistry and stochastic optical reconstruction microscopy (STORM), we show here that Fam65b oligomers form a circumferential ring near the basal taper of the mechanically sensitive stereocilia of murine hair cells. Taperin, a second protein near the taper, forms a dense-core-like structure that is disrupted in the absence of Fam65b. Stereocilia of Fam65b-deficient murine hair cells start to develop, but mechanotransduction is affected and stereocilia deteriorate. Yeast-two-hybrid screens identify RhoC as a Fam65b binding partner. RhoC co-localizes with Fam65b in stereocilia and regulates Fam65b oligomerization. Binding to RhoC and oligomerization are critical for Fam65b function. Our findings thus reveal a highly organized compartment near the base of stereocilia that is critical for hair cell function and affected in disease.https://elifesciences.org/articles/14222hearingdeafnessmechanotransductionsterociliaSTORMFam65b |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Bo Zhao Zizhen Wu Ulrich Müller |
spellingShingle |
Bo Zhao Zizhen Wu Ulrich Müller Murine Fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell function eLife hearing deafness mechanotransduction sterocilia STORM Fam65b |
author_facet |
Bo Zhao Zizhen Wu Ulrich Müller |
author_sort |
Bo Zhao |
title |
Murine Fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell function |
title_short |
Murine Fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell function |
title_full |
Murine Fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell function |
title_fullStr |
Murine Fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell function |
title_full_unstemmed |
Murine Fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell function |
title_sort |
murine fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell function |
publisher |
eLife Sciences Publications Ltd |
series |
eLife |
issn |
2050-084X |
publishDate |
2016-06-01 |
description |
Cochlear hair cells convert sound-induced vibration into electrical signals. FAM65B mutations cause hearing loss by an unknown mechanism. Using biochemistry and stochastic optical reconstruction microscopy (STORM), we show here that Fam65b oligomers form a circumferential ring near the basal taper of the mechanically sensitive stereocilia of murine hair cells. Taperin, a second protein near the taper, forms a dense-core-like structure that is disrupted in the absence of Fam65b. Stereocilia of Fam65b-deficient murine hair cells start to develop, but mechanotransduction is affected and stereocilia deteriorate. Yeast-two-hybrid screens identify RhoC as a Fam65b binding partner. RhoC co-localizes with Fam65b in stereocilia and regulates Fam65b oligomerization. Binding to RhoC and oligomerization are critical for Fam65b function. Our findings thus reveal a highly organized compartment near the base of stereocilia that is critical for hair cell function and affected in disease. |
topic |
hearing deafness mechanotransduction sterocilia STORM Fam65b |
url |
https://elifesciences.org/articles/14222 |
work_keys_str_mv |
AT bozhao murinefam65bformsringlikestructuresatthebaseofstereociliacriticalformechanosensoryhaircellfunction AT zizhenwu murinefam65bformsringlikestructuresatthebaseofstereociliacriticalformechanosensoryhaircellfunction AT ulrichmuller murinefam65bformsringlikestructuresatthebaseofstereociliacriticalformechanosensoryhaircellfunction |
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1721476332857065472 |