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Spongiform Encephal...
Spongiform Encephalopathy in Transgenic Mice Expressing a Point Mutation in the beta 2-alpha 2 Loop of the Prion Protein
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- Sigurdson, Christina J (author)
- University of California San Diego
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- Joshi-Barr, Shivanjali (author)
- University of California San Diego
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- Bett, Cyrus (author)
- University of California San Diego
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- Winson, Olivia (author)
- University of California San Diego
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- Manco, Giuseppe (author)
- University of Vet Medical Vienna
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- Nilsson, Peter (author)
- Linköpings universitet,Kemi,Tekniska fakulteten
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- Margalith, Ilan (author)
- Prionics AG
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- Peretz, David (author)
- University of California San Diego
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- Hornemann, Simone (author)
- Swiss Federal Institute of Technology
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- Wuethrich, Kurt (author)
- Scripps Research Institute
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- Aguzzi, Adriano (author)
- University of Spital Zurich
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(creator_code:org_t)
- Society for Neuroscience, 2011
- 2011
- English.
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In: Journal of Neuroscience. - : Society for Neuroscience. - 0270-6474 .- 1529-2401. ; 31:39, s. 13840-13847
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https://liu.diva-por... (primary) (Raw object)
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
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- Transmissible spongiform encephalopathies are fatal neurodegenerative diseases attributed to misfolding of the cellular prion protein, PrP(C), into a beta-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 beta 2-alpha 2 loop in the NMR structure at pH 4.5 and 20 C, caused transmissible de novo prion disease in transgenic mice. Here we report that expression of mouse PrP with the single-residue substitution D167S, which also results in a structurally well ordered beta 2-alpha 2 loop at 20 degrees C, elicits spontaneous PrP aggregation in vivo. Transgenic mice expressing PrP(D167S) developed a progressive encephalopathy characterized by abundant PrP plaque formation, spongiform change, and gliosis. These results add to the evidence that the beta 2-alpha 2 loop has an important role in intermolecular interactions, including that it may be a key determinant of prion protein aggregation.
Keyword
- TECHNOLOGY
- TEKNIKVETENSKAP
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Sigurdson, Chris ...
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Joshi-Barr, Shiv ...
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Bett, Cyrus
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Winson, Olivia
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Manco, Giuseppe
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Nilsson, Peter
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show more...
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Margalith, Ilan
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Peretz, David
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Hornemann, Simon ...
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Wuethrich, Kurt
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Aguzzi, Adriano
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show less...
- Articles in the publication
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Journal of Neuro ...
- By the university
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Linköping University