Title

The expression of prion protein (PrP(c)) in the megakaryocyte lineage

Authors

Authors

R. Starke; P. Harrison; I. Mackie; G. Wang; J. D. Erusalimsky; R. Gale; J. M. Masse; E. Cramer; A. Pizzey; J. Biggerstaff;S. Machin

Comments

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Abbreviated Journal Title

J. Thromb. Haemost.

Keywords

CD34(+) cells; megakaryocytes; platelets; prion protein; variant; Creutzfeldt-Jacob disease; CREUTZFELDT-JAKOB-DISEASE; NORMAL HUMAN PLATELETS; ACTIVATED PLATELETS; BLOOD-TRANSFUSION; ENDOTHELIAL-CELLS; SCRAPIE; TRANSMISSION; INFECTIVITY; COMPONENTS; RESISTANT; Hematology; Peripheral Vascular Disease

Abstract

Background: Cellular prion protein (Pi-PC) is a naturally occurring protein in normal individuals which adopts an abnormal conformation, termed scrapie prion protein (PrP(Sc)) that is associated with disease. There is great concern that clinically asymptomatic variant Creutzfeldt-Jacob disease (vCJD) may transmit PrPSc in blood transfusion products. PI-PC is widely expressed and has been found in human blood. The majority of cellular borne PrPC is associated with platelets (84%). Although PrPC rnRNA 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 nnegakaryocytes and platelets using electron microscopy; flow cytometry, semiquantitative 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 a-granules and its source is likely to be from megakaryocyte precursors. If PrP(Sc) has a similar distribution, these results have implications for the selection of blood donors and preparation of cell-depleted blood products.

Journal Title

Journal of Thrombosis and Haemostasis

Volume

3

Issue/Number

6

Publication Date

1-1-2005

Document Type

Article

Language

English

First Page

1266

Last Page

1273

WOS Identifier

WOS:000229873700027

ISSN

1538-7933

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