A nonaspan membrane glycoprotein of endoplasmic reticulum, ubiquitously expressed at highest levels in brain, pancreas, heart and insulinoma beta-cell lines, and encoded by WFS1 on 4p16.1
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The disorder takes roots when the Wolframin gene present in our bodies starts mutating.
The mutation results in a loss of Wolframin from the cells that disrupts the production of insulin, causing diabetes.
WFS1 encodes Wolframin, a protein that is defective in individuals suffering from the Wolfram syndrome, characterized by diabetes insipidus, juvenile diabetes, optic atrophy, and deafness.