Usher proteins in inner ear structure and function.

Abstract:

: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.

journal_name

Physiol Genomics

journal_title

Physiological genomics

authors

Ahmed ZM,Frolenkov GI,Riazuddin S

doi

10.1152/physiolgenomics.00135.2013

subject

Has Abstract

pub_date

2013-11-01 00:00:00

pages

987-9

issue

21

eissn

1094-8341

issn

1531-2267

pii

physiolgenomics.00135.2013

journal_volume

45

pub_type

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