PT - JOURNAL ARTICLE AU - Karasik, Avraham AU - O'Hara, Carl AU - Srikanta, S AU - Swift, Michael AU - Soeldner, J Stuart AU - Kahn, C Ronald AU - Herskowitz, Raymonde D TI - Genetically Programmed Selective Islet β-Cell Loss in Diabetic Subjects With Wolfram's Syndrome AID - 10.2337/diacare.12.2.135 DP - 1989 Feb 01 TA - Diabetes Care PG - 135--138 VI - 12 IP - 2 4099 - http://care.diabetesjournals.org/content/12/2/135.short 4100 - http://care.diabetesjournals.org/content/12/2/135.full SO - Diabetes Care1989 Feb 01; 12 AB - 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.