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Haemoglobin Synthesis during Erythroid Maturation in β-Thalassaemia

Nature New Biology volume 240, pages 190–192 (1972)Cite this article

Abstract

In vitro studies of haemoglobin synthesis indicate that unbalanced production of globin chains occurs in β-thalassaemia1,2. More α-chain is always synthesized than non-α-chain, and the inclusion bodies resulting from precipitation of excess α-chain cause ineffective erythropoiesis and a short red cell survival3. There are reports that there is much less chain imbalance in erythroid precursors in the bone marrow of β-thalassaemics than in their reticulocytes4,5, and this may imply either that β-chain mRNA is unstable in β-thalassaemia5 or that there is a compensatory decrease in α-chain synthesis in the marrow6. Since the bulk of haemoglobin (Hb) synthesis occurs in the marrow, the first explanation is not compatible with the existence of the poorly haemoglobinized cells observed in β-thalassaemia, while the second involves a mechanism for which there is no experimental evidence.

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Authors and Affiliations

  1. Department of Haematology and Nuffield Unit of Medical Genetics, University of Liverpool,

    J. B. CLEGG & D. J. WEATHERALL

Authors
  1. J. B. CLEGG
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  2. D. J. WEATHERALL
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CLEGG, J., WEATHERALL, D. Haemoglobin Synthesis during Erythroid Maturation in β-Thalassaemia. Nature New Biology 240, 190–192 (1972). https://doi.org/10.1038/newbio240190a0

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