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Prionscreen


Transmissible spongiform encephalopathies

Development of a blood screning assay for diagnosis of prion diseases in humans

EC contribution
: € 1 602 000
Duration
: 36 months
Starting date
: March 1, 2007
Instrument
: IP
Keywords
: TSE, CJD, biomarker, PMCA, test, blood
Project Number
: SP5A-CT-2007-044438
Web-site
: www.prionscreen.de

Summary:

For many decades, Creutzfeldt-Jakob disease (CJD) was thought to be transmissible among humans only via contact with infectious nervous system tissue. However, recently it was shown that variant Creutzfeldt-Jakob disease (vCJD) is transmissible by blood donation. The transmission of the disease occurred in the incubation period of the donors, which demonstrates that this route is highly efficient. Precautionary measures such as leucodepletion and donor deferral increase the costs of blood and blood products, reduce the donor population, and might lead to shortage of available blood products.


Undetected subclinically infected blood donors bear a great risk for secondary vCJD transmission, persistence and or even spread of vCJD epidemic within the human population. This demonstrates the urgent need for a screening method, which is applicable and repeatable in easily accessible tissues and fluids such as blood.


There is a need to identify new biochemical markers, since conventional tests require brain autopsy or biopsy and surrogate markers are often positive in advanced disease stages only. A test in blood would be more favourable for diagnostic purposes and as a screening assay in blood donors. It needs to reflect disease pathology in the early stages and therefore be able to indicate a prion infection. The tests may be based on PrPSc detection or on surrogate markers reflecting the reaction of the organism to the disease process.


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