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

Diabetes. 1986 Jan;35(1):33-7. doi: 10.2337/diab.35.1.33.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Acanthosis Nigricans / genetics*
  • Acanthosis Nigricans / metabolism
  • Adolescent
  • Adult
  • Blood Glucose / analysis
  • Diseases in Twins
  • Female
  • Glucose Tolerance Test
  • Humans
  • Hyperprolactinemia / genetics
  • Insulin / blood
  • Insulin / metabolism
  • Male
  • Pedigree


  • Blood Glucose
  • Insulin