The physiological role of the PrP protein in normal tissues and fluids

Abstract

Numerous studies have been done to understand howa normal prion protein (PrPc) can be transformed intoa protein resistant to proteases (PrPres) that, whenaccumulated in the central nervous system, induces thecharacteristic alterations of the Prion diseases.However much less attention has been given to understandingthe normal cellular role played by this ubiquitousprotein found in almost all tissues. Several INRAlaboratories have begun to analyse the physiologicalrole of the PrP protein in normal tissues and fluidsusing different models of healthy and infected animals,and also animals and cells with or without expressionof the PrP protein. These studies also address the questionwhether some of the body fluid could also be potentiallyimplicated in the spreading of prion diseases.