Sensitive Quantification of the Disease Associated Prion Protein PrP~(Sc) Using Standard Peptides and Selected Reaction Monitoring

摘要

Prion disease (CJD, BSE) are infectious, fatal neuro-degenerative disorders with no known therapy. The only known constituent of the infectious agent, called prion, is PrPSc. Normal cellular prion protein (PrP~C) is transformed into infectious disease-associated isoform PrPSc. At present, a definite diagnosis of prion disease can only be made after the death of the affected individual by careful analysis of the affected brain tissue. So far all diagnostic tests are antibody-based (WB and ELISA). We are currently developing a MS-based method for the sensitive detection and quantification of PrPSc in a given sample.