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Transmissible spongiform encephalopathies

An integrated immunological and cellular strategy for sensitive TSE diagnosis and strain discrimination

EC contribution
: € 1 750 000
: 36 months
Starting date
: January 1, 2006
: IP
: TSE, epitope typing, surrogate markers, body fluids, scrapie cell assay, immuno-PCR
Project Number
: LSHB-CT-2005-018805


Prion infections result in progressive, fatal neurodegeneration. No effective therapies are available, and medical interventions, possibly including blood transfusions, have resulted in human-to-human transmission of prions. However, no biomarkers are available for preclinical diagnosis of prion infection in body fluids. All approved methods of diagnosis rely exclusively on the detection of the pathological prion protein (PrPSc), which may not be present in all Transmissible spongiform encephalopathies (TSEs).

The TSEUR consortium develops, validates, and exploits innovative reagents and technologies that will address the above problems in three areas: (1) enhanced detection of PrPSc, (2) direct measurement of prion infectivity, and (3) validation of new TSE biomarkers in body fluids.

By using and establishing powerful new panels of picomolar-affinity anti-PrP monoclonal antibodies to a variety of PrP domains, this consortium aims at generating new, highly sensitive tools for TSE diagnosis and strain discrimination. Field tests will validate the partners' recently identified secreted surrogate markers, whose levels are profoundly increased in preclinical prion infections, for identifying potentially contaminated body fluids. Immuno- PCR technology will be explored as a means to enhance the sensitivity threshold of each assay. Finally, the partners will extend the scrapie cell assay technology for rapid and sensitive detection of bona fide prion infectivity in a variety of paradigms.

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