Journal of Clinical Endocrinology & Metabolism, Vol 47, 620-625, Copyright © 1978 by Endocrine Society

ARTICLES

Insulin resistance, acanthosis nigricans, and normal insulin receptors in a young woman: evidence for a postreceptor defect

RS Bar, M Muggeo, J Roth, CR Kahn, J Havrankova and J Imperato-McGinley

We have previously described a group of young females with virilization, acanthosis nigricans, insulin resistance, and markedly decreased binding of insulin to its receptor (syndrome of insulin resistance and acanthosis nigricans type A). The present report concerns a 15-yr-old female with clinical features indistinguishable from the type A patients, including virilization, acanthosis nigricans, and extreme resistance to endogenous and exogenous insulin. Insulin levels were 400-650 microU/ml while fasting and were over 2200 microU/ml when stimulated. Proinsulin was less than 10% of the total immunoassayable insulin. In distinct contrast to the type A patients, insulin receptors on cells from this patient were entirely normal on the basis of specificity, negative cooperativity, affinity, concentration, and interaction with antiinsulin receptor antibodies. These findings suggest the presence of an intracellular defect as the cause of the observed insulin resistance.

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				G Grunberger, Y Zick, and P Gorden Defect in phosphorylation of insulin receptors in cells from an insulin-resistant patient with normal insulin binding Science, March 2, 1984; 223(4639): 932 - 934. [Abstract] [PDF]