In Wolfram syndrome, a rare form of juvenile diabetes, pancreatic β-cell death is not
accompanied by an autoimmune response. Although it has been reported that mutations in
the WFS1 gene are responsible for the development of this syndrome, the precise molecular
mechanisms underlying β-cell death caused by the WFS1 mutations remain unknown. Here
we report that WFS1 is a novel component of the unfolded protein response and has an
important function in maintaining homeostasis of the endoplasmic reticulum (ER) in …