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Familial insulin resistance and acanthosis nigricans. Presence of a postbinding defect.

Authors
  • Schwenk, W F
  • Rizza, R A
  • Mandarino, L J
  • Gerich, J E
  • Hayles, A B
  • Haymond, M W
Type
Published Article
Journal
Diabetes
Publisher
American Diabetes Association
Publication Date
Jan 01, 1986
Volume
35
Issue
1
Pages
33–37
Identifiers
PMID: 3510137
Source
Medline
License
Unknown

Abstract

Type A insulin resistance, associated with acanthosis nigricans and menstrual irregularity, has been ascribed to a decreased concentration of insulin receptors. We now report four affected females from one family, a mother and three daughters (including identical twins) who appear to have the type A syndrome. Two of the kindred had no apparent ovarian dysfunction, while the other two had hyperprolactinemia without other findings of polycystic ovary disease, suggesting a genetic disease with variable penetrance. All had normal erythrocyte and monocyte insulin binding. Insulin dose-response studies to assess glucose metabolism and insulin sensitivity were performed in the affected twins. The dose response to insulin was shifted to the right with a decrease in maximal response. These results are consistent with a postbinding defect in insulin action in these patients.

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