NR ASYA
AU Starke,R.; Harrison,P.; Mackie,I.; Wang,G.; Erusalimsky,J.D.; Gale,R.; Masse,J.M.; Cramer,E.; Pizzey,A.; Biggerstaff,J.; Machin,S.
TI The expression of prion protein (PrPc) in the megakaryocyte lineage
QU Journal of Thrombosis and Haemostasis 2005 Jun; 3(6): 1266-73
PT journal article
AB BACKGROUND: Cellular prion protein (PrPc) is a naturally occurring protein in normal individuals which adopts an abnormal conformation, termed scrapie prion protein (PrPsc) that is associated with disease. There is great concern that clinically asymptomatic variant Creutzfeldt-Jakob disease (vCJD) may transmit PrPsc in blood transfusion products. PrPc is widely expressed and has been found in human blood. The majority of cellular borne PrPc is associated with platelets (84%). Although PrPc mRNA has been demonstrated in platelets, the quantity is unknown and may not reflect the total PrPc present. OBJECTIVE: To investigate the expression of PrPc in the megakaryocyte lineage. METHODS: The expression of PrPc was studied in CD34+ cells, cultured megakaryocytes and platelets using electron microscopy, flow cytometry, semi-quantitative RT-PCR and immunofluorescence confocal microscopy. RESULTS AND CONCLUSIONS: The expression of PrPc appeared to increase with differentiation and polyploidization in the megakaryocyte lineage. PrPc was located within platelet alpha-granules and its source is likely to be from megakaryocyte precursors. If PrPsc has a similar distribution, these results have implications for the selection of blood donors and preparation of cell-depleted blood products.
IN Die Expression des normalen PrPc nimmt bei den 50-150 µm großen Megakaryozyten (Riesenzellen) im Knochenmark mit der Differenzierung (über mehrere Zellteilungen hinweg fortschreitende Reifung zum Megakaryoblasten) und Polyploidisierung (Die Chromosomen und teilweise auch die Zellkerne teilen sich mehrfach ohne anschließende Zellteilung) zu. Wenn sie schließlich in Tausende von kernlosen Thrombozyten (Blutplättchen) zerfallen, dann geht dementsprechend das PrPc auf die Thrombozyten über.
MH Antigens, CD34; Blood Platelets/chemistry; Cell Differentiation; Cell Lineage; Cells, Cultured; Cytoplasmic Granules/chemistry; Hematopoietic Stem Cells/chemistry; Humans; Megakaryocytes/*chemistry/cytology; PrPc Proteins/*analysis/genetics; RNA, Messenger/analysis; Research Support, Non-U.S. Gov't
AD Department of Haematology, University College London, London, UK. r.starke@ucl.ac.uk
SP englisch
PO England