Usher proteins in inner ear structure and function | Zendy

Zubair M. Ahmed | Zendy; Gregory I. Frolenkov | Zendy; Saima Riazuddin | Zendy

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Usher proteins in inner ear structure and function

Author(s) -

Zubair M. Ahmed,

Gregory I. Frolenkov,

Saima Riazuddin

Publication year - 2013

Publication title -

physiological genomics

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.078

H-Index - 112

eISSN - 1531-2267

pISSN - 1094-8341

DOI - 10.1152/physiolgenomics.00135.2013

Subject(s) - stereocilia (inner ear) , biology , inner ear , usher syndrome , kinocilium , transduction (biophysics) , microbiology and biotechnology , function (biology) , cochlea , neuroscience , hair cell , genetics , gene , biochemistry , retinitis pigmentosa

Usher syndrome (USH) is a neurosensory disorder affecting both hearing and vision in humans. Linkage studies of families of USH patients, studies in animals, and characterization of purified proteins have provided insight into the molecular mechanisms of hearing. To date, 11 USH proteins have been identified, and evidence suggests that all of them are crucial for the function of the mechanosensory cells of the inner ear, the hair cells. Most USH proteins are localized to the stereocilia of the hair cells, where mechano-electrical transduction (MET) of sound-induced vibrations occurs. Therefore, elucidation of the functions of USH proteins in the stereocilia is a prerequisite to understanding the exact mechanisms of MET.

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