title: Expression of the cellular prion protein affects posttransfusion recovery and survival of red blood cells in mice.
abstract: BACKGROUND
  Cellular prion protein (PrP(C) ) is expressed on various cell types including red blood cells (RBCs). The PrP(C) plays a key role in the pathogenesis of prion diseases, but its physiologic function remains unclear. PrP(C) is expressed on CD34+ hematopoietic stem cells and its expression is regulated during blood cell differentiation including the erythroid line.
  
  
  METHODS
  We investigated the role of PrP(C) in RBC survival in circulation by transfusing a mix of biotin-labeled RBCs from wild-type (WT) and PrP knockout (KO) mice to groups of recipient mice (WT and KO). The proportion of biotinylated RBCs in peripheral blood was estimated by flow cytometry.
  
  
  RESULTS
  KO RBCs displayed a markedly higher first-day posttransfusion recovery but had a decreased survival in circulation when compared to WT RBCs. Similar results were obtained in all groups of transfused mice, irrespective of RBCs biotinylation level. In addition, we confirmed this finding in an analogous study using Tga20 mice overexpressing PrP(C) and KO mice of a different genetic background.
  
  
  CONCLUSIONS
  Our results demonstrate that PrP(C) expression affects RBC recovery and survival in circulation.
authors.affiliation: Institute of Immunology and Microbiology, First Faculty of Medicine, Charles University, Prague, Czech Republic.
authors.forename: Hana
authors.initials: H
authors.lastname: Glier
authors.affiliation: Laboratory of Cellular Hematology, Center for Biologics Evaluation and Research, Food and Drug Administration, Silver Spring, Maryland.
authors.forename: Jan
authors.initials: J
authors.lastname: Simak
authors.affiliation: Institute of Immunology and Microbiology, First Faculty of Medicine, Charles University, Prague, Czech Republic.
authors.forename: Martin
authors.initials: M
authors.lastname: Panigaj
authors.affiliation: Laboratory of Cellular Hematology, Center for Biologics Evaluation and Research, Food and Drug Administration, Silver Spring, Maryland.
authors.forename: Monique P
authors.initials: MP
authors.lastname: Gelderman
authors.affiliation: Laboratory of Cellular Hematology, Center for Biologics Evaluation and Research, Food and Drug Administration, Silver Spring, Maryland.
authors.forename: Jaroslav G
authors.initials: JG
authors.lastname: Vostal
authors.affiliation: Institute of Immunology and Microbiology, First Faculty of Medicine, Charles University, Prague, Czech Republic.
authors.forename: Karel
authors.initials: K
authors.lastname: Holada
mesh_terms: D000818:Animals; D019921:Biotinylation; D004906:Erythrocyte Count; D017707:Erythrocyte Transfusion; D004912:Erythrocytes / Q000166:cytology* / Q000378:metabolism*; D005260:Female; D005434:Flow Cytometry; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D011328:Prions / Q000378:metabolism / Q000502:physiology*
chemical_list: D011328:Prions