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Schematic representation of the UPR signaling pathways. The UPR is activated by accumulation of unfolded proteins in the ER lumen, followed by BiP dissociation from the three ER stress sensors, i.e., IRE1α, PERK, and ATF6. The UPR instigates a transcriptional and translational response to ER stress in order to restore protein homeostasis. However, upon persistent ER stress, pro-apoptotic signaling is induced and the cell undergoes programmed cell death.
