Spongiform Encephalopathy in Transgenic Mice Expressing a Point Mutation in the β2–α2 Loop of the Prion Protein

This study was supported by the National Institutes of Health (Grants NS055116, NS069566, and U54AI065359 to C.J.S.), the U.S. National Prion Research Program (to C.J.S. and A.A.), the European Union (TSEUR to A.A. and UPMAN to K.W.), the Swiss National Science Foundation, the Novartis Research Foundation (to A.A.), the National Competence Centers for Research on Neural Plasticity and Repair (to A.A.) and on Structural Biology (to K.W.), and ETH Zürich (to K.W.). K.W. is the Cecil H. and Ida M. Green Professor of Structural Biology at The Scripps Research Institute. A.A. is the recipient of an Advanced Investigator Award of the European Research Council. The confocal microscopy experiments were performed at the Human Embryonic Stem Cell Core facility at UC San Diego. We thank Dr. Fred Damberger for illustrating the β2–α2 loop in the NMR structures of the MoPrP¹⁶⁷ and MoPrP, Dr. Michael Oldstone for the 136-158 antibody, and Marianne König, Donna Harclerode, Mona Farahi, Melanie Lucero, and the animal caretakers for technical assistance. The confocal microscopy experiments were performed at the Stem Cell Core Facility at University of California, San Diego.