Tissues of the Laron dwarf are sensitive to insulin-like growth factor I but not to growth hormone

J Clin Endocrinol Metab. 1987 May;64(5):1042-6. doi: 10.1210/jcem-64-5-1042.

Abstract

Tissues from patients with Laron dwarfism are resistant to the actions of endogenous or exogenous GH. As a result, insulin-like growth factor I (IGF-I) levels are low, possibly contributing to the severe growth deficiency that occurs in patients with this syndrome. In this study, we found that erythroid progenitor cells and permanently transformed T-cell lines from two patients with Laron dwarfism responded in vitro to added IGF-I in concentrations ranging between 1-10 ng/mL despite no stimulatory response to added GH in concentrations of up to 500 ng/mL. Normal or near-normal responsiveness to insulin was also demonstrated. The persistence of GH resistance in the cultured T-cell lines confirms the primary genetic nature of the defect in Laron dwarfism. The preservation of in vitro growth responsiveness to IGF-I in hematopoietic tissue from the Laron dwarfs suggests that affected individuals are sensitive to this factor and may respond to it in vivo.

Publication types

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

MeSH terms

  • Adolescent
  • Adult
  • Cell Division / drug effects
  • Cell Transformation, Viral
  • Cells, Cultured
  • Deltaretrovirus
  • Dwarfism / genetics*
  • Dwarfism / pathology
  • Erythrocytes / pathology*
  • Growth Hormone / pharmacology*
  • Hematopoietic Stem Cells / pathology*
  • Humans
  • Insulin-Like Growth Factor I / pharmacology*
  • Male
  • Somatomedins / pharmacology*
  • T-Lymphocytes / pathology*

Substances

  • Somatomedins
  • Insulin-Like Growth Factor I
  • Growth Hormone