Fate of α‐Hemoglobin Chains and Erythrocyte Defects in β‐Thalassemiaa
- 17 December 1990
- journal article
- research article
- Published by Wiley in Annals of the New York Academy of Sciences
- Vol. 612 (1) , 106-117
- https://doi.org/10.1111/j.1749-6632.1990.tb24296.x
Abstract
The fate of alpha-hemoglobin chains and the cause of membrane protein defects in thalassemic erythrocytes have been studied in: (1) human beta-thalassemia syndromes, (2) mouse beta-thalassemia, and (3) normal human erythrocytes loaded with purified alpha-hemoglobin chains. The similarity and differences observed in these three systems underline the importance of insoluble alpha chains and the direct relationship between the amount of these chains and the membrane protein defects. Indeed, in addition to the alpha/non-alpha ratio of globin chain synthesis, the proteolysis and instability of alpha chains are major factors in modulating the cellular defects.Keywords
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