The observations suggest that there may be a number of protein-folding chaperones that play highly significant roles in preservation of ER function in the β -cell to prevent diabetes. β -Cell Failure: Wolfram Syndrome and ER Dysfunction Wolfram syndrome is a rare autosomal-recessive neurodegenerative disorder that is characterized by juvenile-onset diabetes mellitus, optic atrophy and hearing impairment [56] .
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The unfolded protein response is required to maintain the integrity of the endoplasmic reticulum, prevent oxidative stress and preserve differentiation in β-cells.
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