Diminished synthesis of an alpha chain mutant, hemoglobin I (α^{16 lys → glu})

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Abstract

In patients heterozygous for abnormal hemoglobins there is usually less than 50% of the mutant hemoglobin present in peripheral blood. The synthetic rates of α-chain mutants compared to αA have not been reported to date. In this study the production of αA- and αI-chains has been measured in peripheral blood and bone marrow of two patients with approximately 30% hemoglobin I, an α-chain abnormality (α16 lys → glu). The results suggest that the decreased amount of αI compared to αA is due solely to diminished biosynthesis of the αI-chains. The relative rates of synthesis of αI- and αA-chains are similar in both nucleated red cells and reticulocytes indicating that no change occurs during erythroid cell maturation which preferentially affects either αI or αA production.

Authors

G. J. Folayan Esan, F. J. Morgan, J. V. O'Donnell, S. Ford, A. Bank