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

• Main Authors: Bernardo Blanco-Sánchez (Author), Aurélie Clément (Author), Javier Fierro (Author), Philip Washbourne (Author), Monte Westerfield (Author)
• Format: Book
• Published: The Company of Biologists, 2014-05-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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...