Wolfram Syndrome: a rare optic neuropathy in youth with type 1 diabetes.


Wolfram Syndrome (WS) is a rare, autosomal recessive disorder that causes non-autoimmune type 1 diabetes. The etiology involves a single gene mutation of the wolframin protein inducing endoplasmic reticulum stress and apoptosis in selected cell types with resultant diabetes insipidus, diabetes mellitus, optic atrophy, and sensory-neural deafness. Symptoms… (More)
DOI: 10.1097/OPX.0b013e31822f4d8f


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