Defect in messenger RNA for human hemoglobin synthesis in beta thalassemia

Edward J. Benz; Bernard G. Forget

doi:10.1172/JCI106778

^{Division of Hematology, Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115}

^{Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115}

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^{Division of Hematology, Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115}

^{Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115}

Find articles by Forget, B. in: PubMed | Google Scholar

Published in Volume 50, Issue 12 on December 1, 1971
J Clin Invest. 1971;50(12):2755–2760. https://doi.org/10.1172/JCI106778.
© 1971 The American Society for Clinical Investigation

Published December 1, 1971 - Version history

Functional messenger RNA for human hemoglobin synthesis was prepared from reticulocyte lysates of patients with homozygous beta thalassemia and sickle cell anemia. The messenger RNA stimulated the synthesis of human globin chains by a cell-free system derived from Krebs mouse ascites cells. In the presence of beta thalassemia messenger RNA, the system synthesized much less beta chain than alpha chain whereas in the presence of sickle cell anemia messenger RNA, nearly equal amounts of alpha and beta chains were synthesized. The beta/alpha synthetic ratios obtained in the cell-free system were similar to those obtained by incubating intact beta thalassemia and sickle cell anemia reticulocytes in the presence of radioactive leucine. The experiments provide direct evidence of a defect in messenger RNA for beta chains as a cause for the decreased synthesis of beta chains observed in beta thalassemia.

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