RT Journal Article
SR Electronic
T1 Genetically Programmed Selective Islet β-Cell Loss in Diabetic Subjects With Wolfram's Syndrome
JF Diabetes Care
JO Diabetes Care
FD American Diabetes Association
SP 135
OP 138
DO 10.2337/diacare.12.2.135
VO 12
IS 2
A1 Karasik, Avraham
A1 O'Hara, Carl
A1 Srikanta, S
A1 Swift, Michael
A1 Soeldner, J Stuart
A1 Kahn, C Ronald
A1 Herskowitz, Raymonde D
YR 1989
UL http://care.diabetesjournals.org/content/12/2/135.abstract
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.