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Wolfram syndrome protein (WFS1), also called wolframin, is a transmembrane protein, which is located primarily in the endoplasmic reticulum and its expression is induced in response to ER stress, partially through transcriptional activation. ER localization suggests that WFS1 protein has physiological functions in membrane trafficking, secretion, processing and/or regulation of ER calcium homeostasis. It is ubiquitously expressed with highest levels in brain, pancreas, heart, and insulinoma beta-cell lines. Mutations of the WFS1 gene are responsible for two hereditary diseases, autosomal recessive Wolfram syndrome and autosomal dominant low frequency sensorineural hearing loss.