The accumulation of misfolded proteins disrupts the functioning of endoplasmic reticulum (ER), leading to induction of the unfolded protein response (UPR) that protect cells against the toxic buildup of such proteins. However, prolonged stress due to the buildup of these toxic proteins induces specific cell death pathways. There is accumulating evidence implicating ER stress in the development and progression of neurodegenerative diseases. With the improved understanding of the underlying molecular mechanisms, therapeutic interventions that target the ER stress response would be potential strategies to treat neurodegenerative diseases.
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