title: Short survival of phosphatidylserine-exposing red blood cells in murine sickle cell anemia.
abstract: Several transgenic murine models for sickle cell anemia have been developed that closely reproduce the biochemical and physiological disorders in the human disease. A comprehensive characterization is described of hematologic parameters of mature red blood cells, reticulocytes, and red cell precursors in the bone marrow and spleen of a murine sickle cell model in which erythroid cells expressed exclusively human alpha, gamma, and betaS globin. Red cell survival was dramatically decreased in these anemic animals, partially compensated by considerable enhancement in erythropoietic activity. As in humans, these murine sickle cells contain a subpopulation of phosphatidylserine-exposing cells that may play a role in their premature removal. Continuous in vivo generation of this phosphatidylserine-exposing subset may have a significant impact on the pathophysiology of sickle cell disease.
authors.affiliation: Children's Hospital Oakland Research Institute and the Lawrence Berkeley National Laboratory, CA 94609, USA.
authors.forename: K
authors.initials: K
authors.lastname: de Jong
authors.forename: R K
authors.initials: RK
authors.lastname: Emerson
authors.forename: J
authors.initials: J
authors.lastname: Butler
authors.forename: J
authors.initials: J
authors.lastname: Bastacky
authors.forename: N
authors.initials: N
authors.lastname: Mohandas
authors.forename: F A
authors.initials: FA
authors.lastname: Kuypers
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood* / Q000235:genetics / Q000503:physiopathology; D000818:Animals; D019921:Biotinylation; D004905:Erythrocyte Aging; D004910:Erythrocyte Membrane / Q000737:chemistry*; D004912:Erythrocytes / Q000737:chemistry / Q000648:ultrastructure; D004920:Erythropoiesis; D005260:Female; D005434:Flow Cytometry; D005914:Globins / Q000096:biosynthesis / Q000235:genetics; D006451:Hemoglobin, Sickle / Q000096:biosynthesis / Q000235:genetics; D006801:Humans; D008297:Male; D008563:Membrane Lipids / Q000097:blood*; D051379:Mice; D008822:Mice, Transgenic; D023421:Models, Animal; D010100:Oxygen / Q000494:pharmacology; D010718:Phosphatidylserines / Q000097:blood*; D011993:Recombinant Fusion Proteins / Q000096:biosynthesis; D017086:beta-Thalassemia / Q000097:blood / Q000235:genetics
chemical_list: D006451:Hemoglobin, Sickle; D008563:Membrane Lipids; D010718:Phosphatidylserines; D011993:Recombinant Fusion Proteins; D005914:Globins; D010100:Oxygen