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Absence of functional messenger RNA activity for beta globin chain synthesis in beta 0-thalassemia

EJ Benz , PS Swerdlow and BG Forget

Department of Medicine, Children's Hospital Medical Center, Boston, Mass. 02115.

Functional human globin messenger RNA was isolated from reticulocytes of two patients with homozygous beta 0-thalassemia, three patients with sickle cell beta 0-thalassemia, and one patient doubly heterozygous for beta 0-thalassemia and hemoglobin Lepore. When incubated in the Krebs type II mouse ascites tumor-cell-free system, messenger RNA from these patients actively directed the synthesis of human beta s and/or alpha- and gamma-globin chains but failed to stimulate the synthesis of any beta A-chains, even though nonthalassemic human globin mRNA preparations consistently stimulated two to four times as much beta A- or beta S-globin chain synthesis as alpha-chain synthesis when incubated in the same system under the same conditions. These results strongly suggest that functional beta A-chain-specific globin mRNA is absent in beta 0-thalassemia.

Volume 45, Issue 1, pp. 1-10, 01/01/1975
Copyright © 1975 by The American Society of Hematology


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  Copyright © 1975 by American Society of Hematology         Online ISSN: 1528-0020