Figure 2.

Therapeutically targeting the unfolded protein response. Theoretically the unfolded protein response (UPR) could be modified using three main approaches. First, drugs that can stimulate proteasomal degradation and/or autophagy could reduce the mutant protein load, reduce ER stress, and reduce secretion of mutant protein. A second approach could be to support mutant protein folding by upregulating endogenous chaperones, or treating with chemical chaperones, leading to increased secretion of correctly folded protein. Modifying downstream signaling consequences of the UPR is a third approach that could alleviate the ER stress‐induced pathology.