نتایج جستجو برای: stereocilia

تعداد نتایج: 641  

Journal: :The Hearing Journal 2017

Journal: :Development 1998
T Self M Mahony J Fleming J Walsh S D Brown K P Steel

The mouse shaker-1 locus, Myo7a, encodes myosin VIIA and mutations in the orthologous gene in humans cause Usher syndrome type 1B or non-syndromic deafness. Myo7a is expressed very early in sensory hair cell development in the inner ear. We describe the effects of three mutations on cochlear hair cell development and function. In the Myo7a816SB and Myo7a6J mutants, stereocilia grow and form row...

Journal: :Journal of theoretical biology 1998
W A Svrcek-Seiler I C Gebeshuber F Rattay T S Biro H Markum

Brownian motion of the hairs (stereocilia) of amphibian hair cells has been shown in experiments to be in the range of some nm. Our models of the Brownian motion of coupled harmonic oscillators with mechanical properties of stereocilia lead to similar displacements. Computer simulation shows that stochastic fluctuations enhance the encoding of low level acoustic signals. Stochastic resonance lo...

2015
Yuan-Hsiang Lin Giun-Yi Hung Liang-Chun Wu Sheng-Wen Chen Li-Yih Lin Jiun-Lin Horng

The anion exchanger (AE) plays critical roles in physiological processes including CO2 transport and volume regulation in erythrocytes and acid-base regulation in renal tubules. Although expression of the AE in inner-ear hair cells was reported, its specific localization and function are still unclear. Using in situ hybridization, we found that the AE1b transcript is expressed in lateral-line h...

Journal: :PLoS Genetics 2008
Ronna Hertzano Ella Shalit Agnieszka K. Rzadzinska Amiel A. Dror Lin Song Uri Ron Joshua T. Tan Alina Starovolsky Shitrit Helmut Fuchs Tama Hasson Nir Ben-Tal H. Lee Sweeney Martin Hrabe de Angelis Karen P. Steel Karen B. Avraham

Myosin VI, found in organisms from Caenorhabditis elegans to humans, is essential for auditory and vestibular function in mammals, since genetic mutations lead to hearing impairment and vestibular dysfunction in both humans and mice. Here, we show that a missense mutation in this molecular motor in an ENU-generated mouse model, Tailchaser, disrupts myosin VI function. Structural changes in the ...

Journal: :American journal of human genetics 2010
Hana Odeh Kristina L Hunker Inna A Belyantseva Hela Azaiez Matthew R Avenarius Lili Zheng Linda M Peters Leona H Gagnon Nobuko Hagiwara Michael J Skynner Murray H Brilliant Nicholas D Allen Saima Riazuddin Kenneth R Johnson Yehoash Raphael Hossein Najmabadi Thomas B Friedman James R Bartles Richard J H Smith David C Kohrman

Recessive mutations at the mouse pirouette (pi) locus result in hearing loss and vestibular dysfunction due to neuroepithelial defects in the inner ear. Using a positional cloning strategy, we have identified mutations in the gene Grxcr1 (glutaredoxin cysteine-rich 1) in five independent allelic strains of pirouette mice. We also provide sequence data of GRXCR1 from humans with profound hearing...

2017
I-Mei Yu Vicente J Planelles-Herrero Yannick Sourigues Dihia Moussaoui Helena Sirkia Carlos Kikuti David Stroebel Margaret A Titus Anne Houdusse

Cadherin linkages between adjacent stereocilia and microvilli are essential for mechanotransduction and maintaining their organization. They are anchored to actin through interaction of their cytoplasmic domains with related tripartite complexes consisting of a class VII myosin and adaptor proteins: Myo7a/SANS/Harmonin in stereocilia and Myo7b/ANKS4B/Harmonin in microvilli. Here, we determine h...

2018
Yi Li Shuping Jia Huizhan Liu Tomoko Tateya Weiwei Guo Shiming Yang Kirk W. Beisel David Z. Z. He

Citation: Li Y, Jia S, Liu H, Tateya T, Guo W, Yang S, Beisel KW and He DZZ (2018) Characterization of Hair Cell-Like Cells Converted From Supporting Cells After Notch Inhibition in Cultures of the Organ of Corti From Neonatal Gerbils. Front. Cell. Neurosci. 12:73. doi: 10.3389/fncel.2018.00073 The senses of hearing and balance depend upon hair cells, the sensory receptors of the inner ear. Hai...

Journal: :Developmental biology 1986
L G Tilney D J DeRosier

In 8-day-old embryos stereocilia can be identified on the hair cells of the chick cochlea; within each is a small population of actin filaments which extend from the tip of the stereocilium to the apical cytoplasm of the cell. These filaments are not ordered in a regular way, however, and tend to be found near the lateral margins of the stereocilia with large spaces between adjacent filaments. ...

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