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Original Articles

Genetically Programmed Selective Islet β-Cell Loss in Diabetic Subjects With Wolfram's Syndrome

  1. Avraham Karasik, MD,
  2. Carl O'Hara, MD,
  3. S Srikanta, MD,
  4. Michael Swift, MD,
  5. J Stuart Soeldner, MD,
  6. C Ronald Kahn, MD and
  7. Raymonde D Herskowitz, MD
  1. Joslin Clinic and the E.P. Joslin Research Laboratory, Joslin Diabetes Center, Department of Medicine, Brigham and Women's Hospital, New England Deaconess Hospital, Harvard Medical School Boston, Massachusetts Department of Medicine, University of North Carolina Chapel Hill, North Carolina
  1. Address correspondence and reprint requests to R.D. Herskowitz, MD, Joslin Diabetes Center, One Joslin Place, Boston, MA 02215.
Diabetes Care 1989 Feb; 12(2): 135-138. https://doi.org/10.2337/diacare.12.2.135
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Abstract

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.

  • Copyright © 1989 by the American Diabetes Association

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February 1989, 12(2)
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Genetically Programmed Selective Islet β-Cell Loss in Diabetic Subjects With Wolfram's Syndrome
Avraham Karasik, Carl O'Hara, S Srikanta, Michael Swift, J Stuart Soeldner, C Ronald Kahn, Raymonde D Herskowitz
Diabetes Care Feb 1989, 12 (2) 135-138; DOI: 10.2337/diacare.12.2.135

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Genetically Programmed Selective Islet β-Cell Loss in Diabetic Subjects With Wolfram's Syndrome
Avraham Karasik, Carl O'Hara, S Srikanta, Michael Swift, J Stuart Soeldner, C Ronald Kahn, Raymonde D Herskowitz
Diabetes Care Feb 1989, 12 (2) 135-138; DOI: 10.2337/diacare.12.2.135
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