For many decades, 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 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 m ight lead to shortage of available blood products.
From the public health, undetected sub-clinically infected blood donors bear a great risk for secondary vCJD transmission, persistence and or even spread of vCJD epidemic within 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 marker, since conventional tests require brain autopsy or biopsy and surrogate marker 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 early stages and therefore be capable to indicate a prion infection.
The tests may be based on PrPSc detection or on surrogate markers reflecting the reaction of the organism onto the disease process.
The development of such assays requires the study of cell culture systems and animal experiments under controlled conditions. They have to be verified on human biological samples, which are collected on early disease stages.
The goal is the development of a test using multimodal approaches such as detection of disease-specific early markers or abnormal protein aggregation.
This work will be directed towards the identification of specific interacting partners on a protein, mRNA and DNA level.
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