Erythroid phosphatidyl serine exposure is not predictive of thrombotic risk in mice with hemolytic anemia.

Document Type


Publication Date



Anemia-Hemolytic, Animal, Animals-Newborn, Annexin-V, Cell-Size, Erythrocyte-Membrane, Erythrocytes, Flow-Cytometry, Fluorescein-5-isothiocyanate, Lipid-Bilayers, Mice, Mice-Inbred-C57BL, Mice-Mutant-Strains, Phosphatidylserines, Spectrin, Thrombosis

First Page


Last Page


JAX Source

Blood Cells Mol Dis 2000 Feb; 26(1):75-83.


CA34193/CA/NCI, F32DK09482/DK/NIDDK, R01HL29305/HL/NHLBI


Thrombosis is a major complication of human hemolytic anemias such as sickle cell disease, thalassemia, and severe hereditary spherocytosis (HS). Mice with severe HS and severe hereditary elliptocytosis (HE) also suffer from thrombosis, with incidences ranging from 15 and 22% in beta-spectrin- and ankyrin-deficient mice, respectively, to 85 to 100% in alpha-spectrin-deficient and band 3 knockout mice. A contributing factor to thrombosis could be loss of phospholipid asymmetry of the mutant red blood cells (RBCs), with concomitant exposure of the aminophospholipid phosphatidylserine (PS). Increased PS exposure occurs in RBCs from sickle cell and thalassemia patients and in RBCs from band 3-deficient mice. To determine if increased PS exposure correlates with thrombotic risk in HS and HE mice with ankyrin, beta-spectrin, and alpha-spectrin deficiencies, measurements of FITC-labeled annexin V binding to externalized PS on RBCs were performed. PS exposure is elevated in all mice with HS and HE, but the percentage of RBCs with exposed PS does not correlate with thrombotic risk in these mice. Copyright 2000 Academic Press.

Please contact the Joan Staats Library for information regarding this document.