Macroautophagy is regulated by the UPR-mediator CHOP and accentuates the phenotype of SBMA mice.

Altered protein homeostasis underlies degenerative diseases triggered by misfolded proteins, including spinal and bulbar muscular atrophy (SBMA), a neuromuscular disorder caused by a CAG/glutamine expansion in the androgen receptor. Here we show that the unfolded protein response (UPR), an ER protei...

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Bibliographic Details
Main Authors: Zhigang Yu, Adrienne M Wang, Hiroaki Adachi, Masahisa Katsuno, Gen Sobue, Zhenyu Yue, Diane M Robins, Andrew P Lieberman
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2011-10-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC3192827?pdf=render