Experimental transfusion of variant CJD-infected blood reveals previously uncharacterised prion disorder in mice and macaque

Nature Communications
Emmanuel E ComoyJean-Philippe Deslys

Abstract

Exposure of human populations to bovine spongiform encephalopathy through contaminated food has resulted in <250 cases of variant Creutzfeldt-Jakob disease (vCJD). However, more than 99% of vCJD infections could have remained silent suggesting a long-term risk of secondary transmission particularly through blood. Here, we present experimental evidence that transfusion in mice and non-human primates of blood products from symptomatic and non-symptomatic infected donors induces not only vCJD, but also a different class of neurological impairments. These impairments can all be retransmitted to mice with a pathognomonic accumulation of abnormal prion protein, thus expanding the spectrum of known prion diseases. Our findings suggest that the intravenous route promotes propagation of masked prion variants according to different mechanisms involved in peripheral replication.

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Citations

Mar 6, 2018·Brain Pathology·Patricia Aguilar-CalvoChristina J Sigurdson
Jul 5, 2019·Expert Opinion on Drug Discovery·Fabio ModaGiuseppe Legname
Apr 3, 2018·Journal of Neuropathology and Experimental Neurology·Brian S ApplebyMark L Cohen
Jul 7, 2020·EFSA Journal·UNKNOWN EFSA Panel on Biological Hazards (BIOHAZ)Marion M Simmons
Oct 8, 2020·Acta Neuropathologica·Jacqueline MikolEmmanuel Comoy

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Methods Mentioned

BETA
Fluorescence
ELISA
transgenic

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