| abstract |
The present invention provides a novel PrP protein and nucleic acids encoding that protein; where the PrP protein is characterized in vivo by: 1) incomplete glycosylation in relation to the glycosylation of wild-type PrPc; and 2) appropriate cell location, that is, the ability to be transported to the cell surface. This novel, subglycosylated PrP, unlike its normal cellular counterpart, can easily be converted to a protease resistant isoform by incubation with infectious prions. The invention additionally provides systems for the study of prion disorders and methods for using those systems, for example, the study of mechanical processes in the progression of prion-mediated disease or the identification of new therapeutic agents for the treatment of mediated disorders. for prions. In these systems, de-glycosylated, protease resistant, de novo PrP is generated and can be detected by common immunoblotting techniques. |