These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.
3. Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. Hsiao KK; Groth D; Scott M; Yang SL; Serban H; Rapp D; Foster D; Torchia M; Dearmond SJ; Prusiner SB Proc Natl Acad Sci U S A; 1994 Sep; 91(19):9126-30. PubMed ID: 7916462 [TBL] [Abstract][Full Text] [Related]
4. Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. Telling GC; Haga T; Torchia M; Tremblay P; DeArmond SJ; Prusiner SB Genes Dev; 1996 Jul; 10(14):1736-50. PubMed ID: 8698234 [TBL] [Abstract][Full Text] [Related]
5. A Drosophila model of GSS syndrome suggests defects in active zones are responsible for pathogenesis of GSS syndrome. Choi JK; Jeon YC; Lee DW; Oh JM; Lee HP; Jeong BH; Carp RI; Koh YH; Kim YS Hum Mol Genet; 2010 Nov; 19(22):4474-89. PubMed ID: 20829230 [TBL] [Abstract][Full Text] [Related]
6. Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein. Asante EA; Linehan JM; Smidak M; Tomlinson A; Grimshaw A; Jeelani A; Jakubcova T; Hamdan S; Powell C; Brandner S; Wadsworth JD; Collinge J PLoS Pathog; 2013; 9(9):e1003643. PubMed ID: 24086135 [TBL] [Abstract][Full Text] [Related]
7. Molecular biology and genetics of prion diseases. Prusiner SB Philos Trans R Soc Lond B Biol Sci; 1994 Mar; 343(1306):447-63. PubMed ID: 7913765 [TBL] [Abstract][Full Text] [Related]
8. Prion encephalopathies of animals and humans. Prusiner SB Dev Biol Stand; 1993; 80():31-44. PubMed ID: 8270114 [TBL] [Abstract][Full Text] [Related]
9. Accelerated accumulation of misfolded prion protein and spongiform degeneration in a Drosophila model of Gerstmann-Sträussler-Scheinker syndrome. Gavin BA; Dolph MJ; Deleault NR; Geoghegan JC; Khurana V; Feany MB; Dolph PJ; Supattapone S J Neurosci; 2006 Nov; 26(48):12408-14. PubMed ID: 17135402 [TBL] [Abstract][Full Text] [Related]
11. Gerstmann-Sträussler-Scheinker disease and "anchorless prion protein" mice share prion conformational properties diverging from sporadic Creutzfeldt-Jakob disease. Zanusso G; Fiorini M; Ferrari S; Meade-White K; Barbieri I; Brocchi E; Ghetti B; Monaco S J Biol Chem; 2014 Feb; 289(8):4870-81. PubMed ID: 24398683 [TBL] [Abstract][Full Text] [Related]
12. Allelic origin of protease-sensitive and protease-resistant prion protein isoforms in Gerstmann-Sträussler-Scheinker disease with the P102L mutation. Monaco S; Fiorini M; Farinazzo A; Ferrari S; Gelati M; Piccardo P; Zanusso G; Ghetti B PLoS One; 2012; 7(2):e32382. PubMed ID: 22384235 [TBL] [Abstract][Full Text] [Related]
13. Protease resistance of infectious prions is suppressed by removal of a single atom in the cellular prion protein. Leske H; Hornemann S; Herrmann US; Zhu C; Dametto P; Li B; Laferriere F; Polymenidou M; Pelczar P; Reimann RR; Schwarz P; Rushing EJ; Wüthrich K; Aguzzi A PLoS One; 2017; 12(2):e0170503. PubMed ID: 28207746 [TBL] [Abstract][Full Text] [Related]
14. Prion protein transgenes and the neuropathology in prion diseases. DeArmond SJ; Prusiner SB Brain Pathol; 1995 Jan; 5(1):77-89. PubMed ID: 7767493 [TBL] [Abstract][Full Text] [Related]
15. Sc237 hamster PrPSc and Sc237-derived mouse PrPSc generated by interspecies in vitro amplification exhibit distinct pathological and biochemical properties in tga20 transgenic mice. Yoshioka M; Imamura M; Okada H; Shimozaki N; Murayama Y; Yokoyama T; Mohri S Microbiol Immunol; 2011 May; 55(5):331-40. PubMed ID: 21362027 [TBL] [Abstract][Full Text] [Related]
16. Small ruminant nor98 prions share biochemical features with human gerstmann-sträussler-scheinker disease and variably protease-sensitive prionopathy. Pirisinu L; Nonno R; Esposito E; Benestad SL; Gambetti P; Agrimi U; Zou WQ PLoS One; 2013; 8(6):e66405. PubMed ID: 23826096 [TBL] [Abstract][Full Text] [Related]
18. Pathogenic mutations within the hydrophobic domain of the prion protein lead to the formation of protease-sensitive prion species with increased lethality. Coleman BM; Harrison CF; Guo B; Masters CL; Barnham KJ; Lawson VA; Hill AF J Virol; 2014 Mar; 88(5):2690-703. PubMed ID: 24352465 [TBL] [Abstract][Full Text] [Related]
19. Prion seeding activities of mouse scrapie strains with divergent PrPSc protease sensitivities and amyloid plaque content using RT-QuIC and eQuIC. Vascellari S; Orrù CD; Hughson AG; King D; Barron R; Wilham JM; Baron GS; Race B; Pani A; Caughey B PLoS One; 2012; 7(11):e48969. PubMed ID: 23139828 [TBL] [Abstract][Full Text] [Related]
20. A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles. Mercer RCC; Daude N; Dorosh L; Fu ZL; Mays CE; Gapeshina H; Wohlgemuth SL; Acevedo-Morantes CY; Yang J; Cashman NR; Coulthart MB; Pearson DM; Joseph JT; Wille H; Safar JG; Jansen GH; Stepanova M; Sykes BD; Westaway D PLoS Pathog; 2018 Jan; 14(1):e1006826. PubMed ID: 29338055 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]