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Decreased half‐life of insulin‐like growth factor I in Rabson–Mendenhall syndrome
Author(s) -
Longo N.,
Singh R.,
Elsas L. J.
Publication year - 2001
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1023/a:1012411709972
Subject(s) - endocrinology , medicine , insulin , insulin like growth factor , insulin receptor , growth factor , receptor , biology , insulin resistance
Rabson–Mendenhall syndrome is an autosomal recessive disorder of insulin signalling caused by mutations in the insulin receptor gene. Affected patients are insensitive to exogenous insulin. Insulin‐like growth factor I (IGF‐I), whose receptor is similar to the one for insulin and is not impaired in this condition, is not always effective in these patients. To understand the reason for this failure, IGF‐I concentrations were measured in a patient after subcutaneous injection of 0.1 and 0.2 mg/kg of rhIGF‐I. IGF‐I concentrations increased only transiently because of the short half‐life (1.3–3 h, compared to a normal range of 17–22 h). No correlation was found between IGF‐I concentrations and glucose or insulin concentrations. The short half‐life of IGF‐I may contribute to treatment failure in patients with inherited insulin‐resistant syndromes.