The Unfolded Protein Response in Health and Disease

The endoplasmic reticulum (ER) is the principal cellular organelle in which correct folding and maturation of transmembrane, secretory, and ER-resident proteins occur. Research over the past decade has demonstrated that mutations in proteins or agents/conditions that disrupt protein folding adversely affect ER homeostasis, leading to ER stress. This in turn initiates the unfolded protein response (UPR), an integrated intracellular signalling …