Spongiform Encephalopathy in Transgenic Mice Expressing a Point Mutation in the beta 2-alpha 2 Loop of the Prion Protein
نویسندگان
چکیده
Christina J Sigurdson, Shivanjali Joshi-Barr, Cyrus Bett, Olivia Winson, Giuseppe Manco, Peter Nilsson, Ilan Margalith, David Peretz, Simone Hornemann, Kurt Wuethrich and Adriano Aguzzi, Spongiform Encephalopathy in Transgenic Mice Expressing a Point Mutation in the beta 2-alpha 2 Loop of the Prion Protein, 2011, Journal of Neuroscience, (31), 39, 13840-13847. http://dx.doi.org/10.1523/JNEUROSCI.3504-11.2011 Licensee: Society for Neuroscience http://www.sfn.org/
منابع مشابه
Spongiform encephalopathy in transgenic mice expressing a point mutation in the β2-α2 loop of the prion protein.
Transmissible spongiform encephalopathies are fatal neurodegenerative diseases attributed to misfolding of the cellular prion protein, PrP(C), into a β-sheet-rich, aggregated isoform, PrP(Sc). We previously found that expression of mouse PrP with the two amino acid substitutions S170N and N174T, which result in high structural order of the β2-α2 loop in the NMR structure at pH 4.5 and 20°C, cau...
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