Úloha buněčného prionového proteinu v erytroidní diferenciaci

Panigaj, Martin

4 Abstract The cellular prion protein (PrPC ) is evolutionary conserved protein expressed in cells of various origins. Although PrPC plays a basic role in the pathogenesis of the fatal neurodegenerative prion disorders, its physiological role remains enigmatic. Prion diseases are characteristic by long latency period during which they are not identifiable by any conventional methods. Although the blood is an ideal material for developing of screening tests, little is known about traits of PrPC and its role in blood cells. We showed that human erythrocytes express low amounts of PrPC per cell, but due to the high numbers of erythrocytes, they are major contributors to the pool of blood cell-associated PrPC . Based on our biochemical characterization we propose that PrPC on human erythrocytes is uniquely modified. Such a modification in abnormal prion protein may complicate screening tests for prion diseases in blood. It was reported that prion diseases deregulate the transcription of erythroid genes, and PrP-/- mice demonstrate a defective response to experimental anemia. To investigate the role of the PrPC in erythropoiesis, we studied the protein's expression on mouse erythroid precursors in vivo and in vitro. We showed that surface expression of PrPC on erythroid precursors in bone marrow and spleen...

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