Comparison of Bovine BSE-L and Human CJD prions phenotypic traits upon transmission To Human PrP transgenic mice
- P.I. Vincent Béringue et Hubert Laude, INRA, Jouy en Josas, France
- Stéphane Haïk, INSERM UMRS975, Paris, France
- Armand Perret-Liaudet, CHU Lyon, Lyon, France
2008: 1 year / Grant: 70 000 euros
Project: It is now well established that vCJD is due to BSE exposure through contaminated food.
However atypical forms of BSE such as BSE-L and BSE-H have been discovered and have been
considered as sporadic BSE strains. Since many clues seem to converge to suggest that sporadic
CJD- type 2a could be due L-BSE, the aim of“Bohutohu” wasto decipher the potential link between
L-BSE and human prion diseases.
Main results and related published data: Transgenic mice expressing human PRNP were
inoculated with known sCJD (17 cases) and L-BSE infected brain homogenates. Iterative subpassages
were performed (3 or 4 times). Even if similar strain PrPTSE signatures with cortical MM2-sCJD
prions(resistance to guanidinium chloride treatment, lymphotropism, and deposition) were observed,
there was no evidence of L-BSE responsibility in the etiology of the known form of sCJD, since the
incubation periods and the differential resistance to PK digestion were notcompatible. However, as
this study revealed, there was no significant barrier of transmission of L-BSE in transgenic mice
expressing human PrP, it appearsstill important to maintain surveillance and measuresto avoid food
contamination by this L-BSE agent.
- Transmission of uncommon forms of bovine prions to transgenic mice expressing human PrP: questions and progress. Béringue V. Prion 2009 (Thessalonique, September 2009).
- L-Type and Epizootic BSEs: Playing the Cat and Mouse game. Béringue V. Prion 2011(Montreal, May 2011).
- Prion diversity and evolution: what animal strain typing tells us. Béringue V. Prion 2012
(Amsterdam, May 2012).
- Facilitated cross-species transmission of prions in extraneural tissue. Béringue V, Herzog
L, Jaumain E, Reine F, Sibille P, Le Dur A, Vilotte JL, Laude H. Science. 2012 Jan 27;335(6067):472-
5. doi: 10.1126/science.1215659.
- Absence of Evidence for a Causal Link between Bovine Spongiform Encephalopathy Strain
Variant L-BSE and Known Forms of Sporadic Creutzfeldt-Jakob Disease in Human PrP
Transgenic Mice. Jaumain E, Quadrio I, Herzog L, Reine F, Rezaei H, Andréoletti O,Laude H, PerretLiaudet
A, Haïk S, Béringue V. J Virol. 2016 Nov 14;90(23):10867-10874. Print 2016 Dec 1.
- Emergence of two prion subtypes in ovine PrP transgenic mice infected with human MM2-
cortical Creutzfeldt-Jakob disease prions. Chapuis J, Moudjou M, Reine F, Herzog L, Jaumain
E, Chapuis C, Quadrio I, Boulliat J, Perret-Liaudet A, Dron M, Laude H, Rezaei H, Béringue V. Acta
Neuropathol Commun. 2016 Feb 5;4:10. doi: 10.1186/s40478-016-0284-9.