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Model for the role of endoplasmic reticulum (ER) stress in neurodegeneration. In addition to rare inherited mutations in a single protein that disrupt its proper folding, degenerating neurons are exposed to numerous other insults (e.g., oxidative stress, inflammation, metabolic disturbances) that can compromise protein folding and lead to ER stress. Abbreviation: UPR, unfolded protein response. Modified with permission from Reference 149. Copyright © 2014 by Elsevier.
