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Science 17 January 1997:
Vol. 275. no. 5298, pp. 402 - 404
DOI: 10.1126/science.275.5298.402

Reports

Transmission of the BSE Agent to Mice in the Absence of Detectable Abnormal Prion Protein

Corinne I. Lasmézas, * Jean-Philippe Deslys, Olivier Robain, Alexandre Jaegly, Vincent Beringue, Jean-Michel Peyrin, Jean-Guy Fournier, Jean-Jacques Hauw, Jean Rossier, Dominique Dormont

The agent responsible for transmissible spongiform encephalopathies (TSEs) is thought to be a malfolded, protease-resistant version (PrPres) of the normal cellular prion protein (PrP). The interspecies transmission of bovine spongiform encephalopathy (BSE) to mice was studied. Although all of the mice injected with homogenate from BSE-infected cattle brain exhibited neurological symptoms and neuronal death, more than 55 percent had no detectable PrPres. During serial passage, PrPres appeared after the agent became adapted to the new host. Thus, PrPres may be involved in species adaptation, but a further unidentified agent may actually transmit BSE.

C. I. Lasmézas, J.-P. Deslys, A. Jaegly, V. Beringue, J.-M. Peyrin, D. Dormont, Commissariat à l'Energie Atomique, Service de Neurovirologie, DSV/DRM/SSA, B.P. 6, 60-68 avenue du General Leclerc, 92265 Fontenay-aux-Roses Cedex, France.
O. Robain, Hôpital Saint Vincent de Paul, INSERM U 29, 74 avenue Denfert-Rochereau, 75674 Paris Cedex 14, France.
J.-G. Fournier, Hôpital de la Salpétrière, INSERM U 153, 47 boulevard de l'Hôpital, 75651 Paris Cedex 13, France.
J.-J. Hauw, Hôpital de la Salpétrière, Laboratoire Escourolle, INSERM U 360, 47 boulevard de l'Hôpital, 75651 Paris Cedex 13, France.
J. Rossier, Ecole Supérieure de Physique et Chimie Industrielles, CNRS URA 2054, 10 rue Vauquelin, 75231 Paris Cedex 5, France.
*   To whom correspondence should be addressed. E-mail: CORINNE.LASMEZAS{at}cea.fr


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