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Research Article Free access | 10.1172/JCI106993

Equal synthesis of α- and β-globin chains in erythroid precursors in heterozygous β-thalassemia

Yuet Wai Kan, David G. Nathan, and Harvey F. Lodish

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

Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Department of Biology, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139

Find articles by Kan, Y. in: PubMed | Google Scholar

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

Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Department of Biology, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139

Find articles by Nathan, D. in: PubMed | Google Scholar

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

Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Department of Biology, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139

Find articles by Lodish, H. in: PubMed | Google Scholar

Published July 1, 1972 - More info

Published in Volume 51, Issue 7 on July 1, 1972
J Clin Invest. 1972;51(7):1906–1909. https://doi.org/10.1172/JCI106993.
© 1972 The American Society for Clinical Investigation
Published July 1, 1972 - Version history
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Abstract

In patients with heterozygous β-thalassemia, the β/α synthetic ratio in marrow erythroid cells incubated in vitro is 1, whereas in reticulocytes the ratio is 0.5. These ratios reflect the equal synthesis of the two chains on the polyribosomes of the bone marrow and unequal synthesis on the polyribosomes of the peripheral blood reticulocytes. α- and β-chain synthesis is also equal in marrow cells in vivo. Equal synthesis is probably due both to a decrease in α-chain synthesis and an increase in β-chain synthesis in bone marrow erythroid cells and may contribute to the absence of overt hemolysis due to excess α-globin chain accumulation in heterozygous β-thalassemia.

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