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...
Main Authors: | , , , , , , , |
---|---|
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 |