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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 02115Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115Department of Biology, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139

Published July 1972

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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