| 1. |
- Garcia-Pueyo, Lluís, et al.
(författare)
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Integrity 2024: Integrity in Social Networks and Media
- 2024
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Ingår i: WSDM 2024 - Proceedings of the 17th ACM International Conference on Web Search and Data Mining. - : Association for Computing Machinery, Inc. ; , s. 1212-1213
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Konferensbidrag (refereegranskat)abstract
- Integrity 2024 is the fifth edition of the Workshop on Integrity in Social Networks and Media, held in conjunction with the ACM Conference on Web Search and Data Mining (WSDM) since the 2020 edition [1-4]. The goal of the workshop is to bring together academic and industry researchers working on integrity, fairness, trust and safety in social networks to discuss the most pressing risks and cutting-edge technologies to reliably measure and mitigate them. The event consists of invited talks from academic experts and industry leaders as well as peer-reviewed papers and posters through an open call-for-papers.
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| 2. |
- Sigurdson, Christina J, et al.
(författare)
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De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis.
- 2009
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Ingår i: Proceedings of the National Academy of Sciences of the United States of America. - : Proceedings of the National Academy of Sciences. - 0027-8424 .- 1091-6490. ; 106:1, s. 304-309
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Tidskriftsartikel (refereegranskat)abstract
- Most transmissible spongiform encephalopathies arise either spontaneously or by infection. Mutations of PRNP, which encodes the prion protein, PrP, segregate with phenotypically similar diseases. Here we report that moderate overexpression in transgenic mice of mPrP(170N,174T), a mouse PrP with two point mutations that subtly affect the structure of its globular domain, causes a fully penetrant lethal spongiform encephalopathy with cerebral PrP plaques. This genetic disease was reproduced with 100% attack rate by intracerebral inoculation of brain homogenate to tga20 mice overexpressing WT PrP, and from the latter to WT mice, but not to PrP-deficient mice. Upon successive transmissions, the incubation periods decreased and PrP became more protease-resistant, indicating the presence of a strain barrier that was gradually overcome by repeated passaging. This shows that expression of a subtly altered prion protein, with known 3D structure, efficiently generates a prion disease.
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| 3. |
- Sigurdson, Christina J, et al.
(författare)
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Spongiform Encephalopathy in Transgenic Mice Expressing a Point Mutation in the beta 2-alpha 2 Loop of the Prion Protein
- 2011
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Ingår i: Journal of Neuroscience. - : Society for Neuroscience. - 0270-6474 .- 1529-2401. ; 31:39, s. 13840-13847
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Tidskriftsartikel (refereegranskat)abstract
- 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.
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