Complexes of Usher proteins preassemble at the endoplasmic reticulum and are required for trafficking and ER homeostasis

Usher syndrome (USH), the leading cause of hereditary combined hearing and vision loss, is characterized by sensorineural deafness and progressive retinal degeneration. Mutations in several different genes produce USH, but the proximal cause of sensory cell death remains mysterious. We adapted a pro...

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Bibliographic Details
Main Authors: Bernardo Blanco-Sánchez, Aurélie Clément, Javier Fierro, Philip Washbourne, Monte Westerfield
Format: Article
Language:English
Published: The Company of Biologists 2014-05-01
Series:Disease Models & Mechanisms
Subjects:
Online Access:http://dmm.biologists.org/content/7/5/547