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2011 Call for projects in the field of viral safety for biological products
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Selected projects 2009

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Detection of PrPSc (a biomarker for TSE infection) in blood using QUIC shake technology: validation of in vitro studies with infectivity models

PIs : J.C. Manson and S. McCutcheon - The Roslin Institute, Neuropathogenesis Division - UK

Other Investigator : B. Caughey, National Institutes for Allergy and Infectious Diseases, National Institutes of Health, Rocky Mountain Laboratories - USA

Prion diseases are a group of brain disorders that can occur in both humans and animals. Most commonly described examples in animals are BSE, affecting cattle and scrapie in sheep. A human form of prion disease, variant CJD (vCJD), emerged in 1996, reportedly through the consumption of BSE contaminated meat products which had entered the human food chain. Affected individuals can harbour a ‘silent’ vCJD infection for many years before the onset of clinical signs and ultimately death. Additionally, it has been shown that humans are able to pass on vCJD to other humans following blood transfusion.

To date, there have been 4 probable cases of vCJD associated with blood transfusion. The cause of these diseases is thought to be due to the presence of an abnormal form (called PrPSc) of the normal prion protein (called PrPC). At present, there is no easy and reliable means of assessing whether a person is infected with the vCJD agent: current methods rely on post-mortem diagnosis and are either time consuming or technically challenging. Consequently, there is a major requirement for a blood-based assay capable of identifying vCJD infected humans, before the development of obvious clinical symptoms.

We have previously identified a model infection system, namely sheep infected with BSE, which has distinct advantages to others in that PrPSc accumulates in similar tissues to those observed in humans who have vCJD and the blood components can be prepared from BSE-infected sheep to a similar specification to those used in human transfusion medicine. We propose to use previously collected blood material from a large sheep-BSE infection study to investigate the potential to identify and amplify, PrPSc from blood and its component parts.


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