Insulin-producing (5-cells were selectively absent from the islets of Langerhans in postmortem specimens from two patients with Wolfram's syndrome. In families with multiple cases of this syndrome, we found a very high concordance rate (r = .910, P < .001) among siblings for age at onset of diabetes mellitus. Taken together with the lack of markers for an autoimmune process, these findings suggest that diabetes mellitus in this syndrome results from genetically programmed selective p-cell death.
Genetically Programmed Selective Islet β-Cell Loss in Diabetic Subjects With Wolfram's Syndrome
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Avraham Karasik, Carl O'Hara, S Srikanta, Michael Swift, J Stuart Soeldner, C Ronald Kahn, Raymonde D Herskowitz; Genetically Programmed Selective Islet β-Cell Loss in Diabetic Subjects With Wolfram's Syndrome. Diabetes Care 1 February 1989; 12 (2): 135–138. https://doi.org/10.2337/diacare.12.2.135
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