Enhanced erythrocyte apoptosis in sickle cell anemia, thalassemia and glucose-6-phosphate dehydrogenase deficiency.
Lang, Karl S, Roll, Benjamin, Mysina, Svetlana, Schittenhelm, Markus, Scheel-Walter, Hans-Gerhard, Kanz, Lothar, Fritz, Jasmin, Lang, Florian, Huber, Stephan M and Wieder, Thomas
(2002)
Enhanced erythrocyte apoptosis in sickle cell anemia, thalassemia and glucose-6-phosphate dehydrogenase deficiency.
Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology, 12 (5-6).
pp. 365-72.
ISSN 1015-8987
Abstract
Erythrocyte diseases such as sickle cell anemia, thalassemia and glucose-6-phosphate dehydrogenase deficiency decrease the erythrocyte life span, an effect contributing to anemia. Most recently, erythro-cytes have been shown to undergo apoptosis upon increase of cytosolic Ca(2+) activity. The present study has been performed to explore whether sickle cell anemia, thalassemia and glucose-6-phosphate dehydrogenase deficiency enhance the sensitivity of erythrocytes to osmotic shock, oxidative stress or energy depletion, all maneuvers known to increase cytosolic Ca(2+) activity. To this end, annexin binding as an indicator of apoptosis has been determined by FACS analysis. Erythrocytes from healthy individuals, from patients with sickle cell anemia, thalassemia or glucose-6-phosphate dehydrogenase deficiency all responded to osmotic shock (up to 950 mOsm by addition of sucrose for 24 hours), to oxidative stress (up to 1.0 mM tetra-butyl-hydroxyperoxide tBOOH) and to energy depletion (up to 48 hours glucose deprivation) with enhanced annexin binding. However, the sensitivity of sickle cells and of glucose-6-phosphate dehydrogenase deficient cells to osmotic shock and of sickle cells, thalassemic cells and glucose-6-phosphate dehydrogenase deficient cells to oxidative stress and to glucose depletion was significantly higher than that of control cells. Annexin binding was further stimulated by Ca(2+) ionophore ionomycin with significantly higher sensitivity of sickle cells and glucose-6-phosphate dehydrogenase deficient cells as compared to intact cells. In conclusion, sickle cells, thalassemic cells and glucose-6-phosphate dehydrogenase deficient erythrocytes are more sensitive to osmotic shock, oxidative stress and/or energy depletion, thus leading to enhanced apoptosis of those cells. The accelerated apoptosis then contributes to the shortened life span of the defective erythrocytes.
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Date Deposited: 08 Oct 2018 15:22 |
Last Modified: 09 Oct 2018 11:07 |
Contributors
Author: |
Karl S Lang
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Author: |
Benjamin Roll
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Svetlana Mysina
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Markus Schittenhelm
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Hans-Gerhard Scheel-Walter
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Lothar Kanz
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Jasmin Fritz
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Author: |
Florian Lang
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Author: |
Stephan M Huber
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Author: |
Thomas Wieder
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