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Research Article Free access | 10.1172/JCI119666

Removal of erythrocyte membrane iron in vivo ameliorates the pathobiology of murine thalassemia.

P V Browne, O Shalev, F A Kuypers, C Brugnara, A Solovey, N Mohandas, S L Schrier, and R P Hebbel

Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota 55455, USA.

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Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota 55455, USA.

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Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota 55455, USA.

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Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota 55455, USA.

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Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota 55455, USA.

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Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota 55455, USA.

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Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota 55455, USA.

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Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota 55455, USA.

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Published September 15, 1997 - More info

Published in Volume 100, Issue 6 on September 15, 1997
J Clin Invest. 1997;100(6):1459–1464. https://doi.org/10.1172/JCI119666.
© 1997 The American Society for Clinical Investigation
Published September 15, 1997 - Version history
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Abstract

Abnormal deposits of free iron are found on the cytoplasmic surface of red blood cell (RBC) membranes in beta-thalassemia. To test the hypothesis that this is of importance to RBC pathobiology, we administered the iron chelator deferiprone (L1) intraperitoneally to beta-thalassemic mice for 4 wk and then studied RBC survival and membrane characteristics. L1 therapy decreased membrane free iron by 50% (P = 0.04) and concomitantly improved oxidation of membrane proteins (P = 0.007), the proportion of RBC gilded with immunoglobulin (P = 0.001), RBC potassium content (P < 0.001), and mean corpuscular volume (P < 0.001). Osmotic gradient ektacytometry confirmed a trend toward improvement of RBC hydration status. As determined by clearance of RBC biotinylated in vivo, RBC survival also was significantly improved in L1-treated mice compared with controls (P = 0.007). Thus, in vivo therapy with L1 removes pathologic free iron deposits from RBC membranes in murine thalassemia, and causes improvement in membrane function and RBC survival. This result provides in vivo confirmation that abnormal membrane free iron deposits contribute to the pathobiology of thalassemic RBC.

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