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Effect of α‐Chain Precipitates on Bone Marrow Function in Homozygous β‐Thalassaemia
Author(s) -
Wickramasinghe S. N.,
Letsky E.,
Moffatt B.
Publication year - 1973
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1973.tb01721.x
Subject(s) - ineffective erythropoiesis , bone marrow , erythropoiesis , chemistry , microbiology and biotechnology , biochemistry , biology , immunology , medicine , anemia
S ummary . An autoradiographic study of protein synthesis by erythropoietic cells in liomozygous β‐thalassaemia has shown that a high proportion of non‐dividing, late polychromatic erythroblasts fail to become labelled when incubated with radioactive amino acids. It is possible that this abnormality and the previously described disturbance of cell proliferation in the early polychromatic erythroblasts result from damage by intracellular α‐chain precipitates. This possibility has been investigated by correlating the extent of α‐chain precipitation with protein or DNA synthetic activity in individual cells. Over 80% of late polychromatic cells with the largest α‐chain inclusions failed to label with 3 H‐leucine or 3 H‐phenylalanine but 10–23% of cells with no inclusions were also unlabelled. None of the dividing, early polychromatic cells with moderate or large quantities of insoluble α‐chains incorporated 3 H‐thymidine. These results support the hypothesis that a‐chain precipitates are associated with and possibly responsible for the ineffectiveness of erythropoiesis in thalassaemia, provided it is assumed that such precipitates are continuously degraded or extruded. The latter assumption is necessary to account for the presence of several metabolically abnormal cells with no α‐chain precipitates.