This Article Full Text (PDF) Submit a related Letter to the Editor Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by POSTEL-VINAY, M. C. Articles by RAPPAPORT, R. Search for Related Content PubMed PubMed Citation Articles by POSTEL-VINAY, M. C. Articles by RAPPAPORT, R.

No Evidence for a Defect in Growth Hormone Binding to Liver Membranes in Thalassemia Major^*

Unité de Recherches en Biobgie et Pathologie de la Croissance et du Dévebppement and Service d'Hématobgie (R.G.), Hôpital des Enfants-Mabdes Paris, France

Address all correspondence and requests for reprints to: Dr. M. Postel-Vinay, INSERM U.30, Tour Technique 6ème Etage, Hôpital des Enfants-Malades, 149 rue de Sèvres, 75015 Paris, France.

To test the hypothesis of a defect in GH-receptor interaction, which could explain the growth failure of thalassemic children, the binding of [¹²⁵I] human (h) GH tomembrane fractions prepared from liver biopsies was studied. Small amounts of liver were obtained from 6 girls and 11 boys with homozygous β-thalassemia, aged 3–15 yr, all prepubertal, at the time of splenectomy. Specific binding of [¹²⁵I]hGH ranged from 0.37–5.11% of the added radioactivity/100 µgliver membrane protein, with variations in both receptor number and binding affinity. This 14-fold variation in hGH binding to liver membranes of thalassemic children was comparable to that in membrane fractions of livers obtained from normal donors at the time of liver transplant.The binding of insulin to liver membranes from the thalassemic patients ranged from 9.8–17.9% of the added radioactivity/100 ng membrane protein and from 2.8–15.0%/100 µg membrane protein in the normal donors. Insulin and GH binding to liver membranes did not vary in a consistent way. A 3-fold difference was found in 5ô-nucleotidase activity of the membrane fractions. Histological hepatic modifications were assessed with respect to siderosis and fibrosis. No correlation was found between these parameters and GH binding. These results suggest that possible membrane alterations are not the only reason for the variations in hGH binding. All patients had retarded growth, and all but 2 had low plasma insulin-like growth factor I levels. No relationship was found between the level of GH binding to liver membranes and the growth failure. Thus, a defect in GH binding to liver membranes is probably not the cause of the growth retardation of thalassemic children.

^* Presented in part at the 26th Annual Meeting of the European Society for Pediatric Endocrinology, Toulouse, France, September 1987. This work was supported by INSERM.

Received May 11, 1988.

This article has been cited by other articles:

				X. Y. Shen, R. I. G. Holt, J. P. Miell, S. Justice, B. Portmann, M.-C. Postel-Vinay, and R. J. M. Ross Cirrhotic Liver Expresses Low Levels of the Full-Length and Truncated Growth Hormone Receptors J. Clin. Endocrinol. Metab., July 1, 1998; 83(7): 2532 - 2538. [Abstract] [Full Text]

				N. F. Olivieri and G. M. Brittenham Iron-Chelating Therapy and the Treatment of Thalassemia Blood, February 1, 1997; 89(3): 739 - 761. [Full Text] [PDF]