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Blood, 1 December 2008, Vol. 112, No. 12, pp. 4739-4745.
Prepublished online as a Blood First Edition Paper on July 22, 2008; DOI 10.1182/blood-2008-04-152520.
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Submitted April 18, 2008
Accepted June 28, 2008
Prion diseases are efficiently transmitted by blood transfusion in sheep
Fiona Houston*, Sandra McCutcheon, Wilfred Goldmann, Angela Chong, James Foster, Silvia Siso, Lorenzo Gonzalez, Martin Jeffrey, and Nora Hunter
Division of Animal Production and Public Health, Faculty of Veterinary Medicine, University of Glasgow, Glasgow, United Kingdom
Neuropathogenesis Division, Roslin Institute, University of Edinburgh, Edinburgh, United Kingdom
Veterinary Laboratories Agency, Lasswade Laboratory, Edinburgh, United Kingdom
* Corresponding author; email: f.houston{at}vet.gla.ac.uk.
The emergence of variant Creutzfeld-Jakob disease (vCJD), following on from the bovine spongiform encephalopathy (BSE) epidemic, led to concerns about the potential risk of iatrogenic transmission of disease by blood transfusion and the introduction of costly control measures to protect blood supplies. We previously reported preliminary data demonstrating the transmission of BSE and natural scrapie by blood transfusion in sheep. The final results of this experiment, reported here, give unexpectedly high transmission rates by transfusion of 36% for BSE and 43% for scrapie. A proportion of BSE-infected tranfusion recipients (3/8) survived for up to 7 years without showing clinical signs of disease. The majority of transmissions resulted from blood collected from donors at >50% of the estimated incubation period. The high transmission rates and relatively short and consistent incubation periods in clinically positive recipients suggest that infectivity titres in blood were substantial and/or that blood transfusion is an efficient method of transmission. This experiment has established the value of using sheep as a model for studying transmission of vCJD by blood products in humans.

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