282 related articles for article (PubMed ID: 24086135)
1. 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]
2. A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP.
Yang W; Cook J; Rassbach B; Lemus A; DeArmond SJ; Mastrianni JA
J Neurosci; 2009 Aug; 29(32):10072-80. PubMed ID: 19675240
[TBL] [Abstract][Full Text] [Related]
3. Prion proteins with different conformations accumulate in Gerstmann-Sträussler-Scheinker disease caused by A117V and F198S mutations.
Piccardo P; Liepnieks JJ; William A; Dlouhy SR; Farlow MR; Young K; Nochlin D; Bird TD; Nixon RR; Ball MJ; DeCarli C; Bugiani O; Tagliavini F; Benson MD; Ghetti B
Am J Pathol; 2001 Jun; 158(6):2201-7. PubMed ID: 11395398
[TBL] [Abstract][Full Text] [Related]
4. Isolation of infectious, non-fibrillar and oligomeric prions from a genetic prion disease.
Vanni I; Pirisinu L; Acevedo-Morantes C; Kamali-Jamil R; Rathod V; Di Bari MA; D'Agostino C; Marcon S; Esposito E; Riccardi G; Hornemann S; Senatore A; Aguzzi A; Agrimi U; Wille H; Nonno R
Brain; 2020 May; 143(5):1512-1524. PubMed ID: 32303068
[TBL] [Abstract][Full Text] [Related]
5. Mutant PrPSc conformers induced by a synthetic peptide and several prion strains.
Tremblay P; Ball HL; Kaneko K; Groth D; Hegde RS; Cohen FE; DeArmond SJ; Prusiner SB; Safar JG
J Virol; 2004 Feb; 78(4):2088-99. PubMed ID: 14747574
[TBL] [Abstract][Full Text] [Related]
6. Transmission Properties of Human PrP 102L Prions Challenge the Relevance of Mouse Models of GSS.
Asante EA; Grimshaw A; Smidak M; Jakubcova T; Tomlinson A; Jeelani A; Hamdan S; Powell C; Joiner S; Linehan JM; Brandner S; Wadsworth JD; Collinge J
PLoS Pathog; 2015 Jul; 11(7):e1004953. PubMed ID: 26135918
[TBL] [Abstract][Full Text] [Related]
7. 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]
8. Transmissibility of Gerstmann-Sträussler-Scheinker syndrome in rodent models: New insights into the molecular underpinnings of prion infectivity.
Nonno R; Angelo Di Bari M; Agrimi U; Pirisinu L
Prion; 2016 Nov; 10(6):421-433. PubMed ID: 27892798
[TBL] [Abstract][Full Text] [Related]
9. Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS.
Asante EA; Linehan JM; Tomlinson A; Jakubcova T; Hamdan S; Grimshaw A; Smidak M; Jeelani A; Nihat A; Mead S; Brandner S; Wadsworth JDF; Collinge J
PLoS Biol; 2020 Jun; 18(6):e3000725. PubMed ID: 32516343
[TBL] [Abstract][Full Text] [Related]
10. Gerstmann-Sträussler-Scheinker disease subtypes efficiently transmit in bank voles as genuine prion diseases.
Pirisinu L; Di Bari MA; D'Agostino C; Marcon S; Riccardi G; Poleggi A; Cohen ML; Appleby BS; Gambetti P; Ghetti B; Agrimi U; Nonno R
Sci Rep; 2016 Feb; 6():20443. PubMed ID: 26841849
[TBL] [Abstract][Full Text] [Related]
11. Towards authentic transgenic mouse models of heritable PrP prion diseases.
Watts JC; Giles K; Bourkas ME; Patel S; Oehler A; Gavidia M; Bhardwaj S; Lee J; Prusiner SB
Acta Neuropathol; 2016 Oct; 132(4):593-610. PubMed ID: 27350609
[TBL] [Abstract][Full Text] [Related]
12. 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]
13. 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]
14. Homozygous R136S mutation in PRNP gene causes inherited early onset prion disease.
Ximelis T; Marín-Moreno A; Espinosa JC; Eraña H; Charco JM; Hernández I; Riveira C; Alcolea D; González-Roca E; Aldecoa I; Molina-Porcel L; Parchi P; Rossi M; Castilla J; Ruiz-García R; Gelpi E; Torres JM; Sánchez-Valle R
Alzheimers Res Ther; 2021 Oct; 13(1):176. PubMed ID: 34663460
[TBL] [Abstract][Full Text] [Related]
15. Genetic and infectious prion diseases.
Prusiner SB
Arch Neurol; 1993 Nov; 50(11):1129-53. PubMed ID: 8105771
[TBL] [Abstract][Full Text] [Related]
16. 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]
17. A novel PRNP-P105S mutation associated with atypical prion disease and a rare PrPSc conformation.
Tunnell E; Wollman R; Mallik S; Cortes CJ; Dearmond SJ; Mastrianni JA
Neurology; 2008 Oct; 71(18):1431-8. PubMed ID: 18955686
[TBL] [Abstract][Full Text] [Related]
18. Phenotypic variability of Gerstmann-Sträussler-Scheinker disease is associated with prion protein heterogeneity.
Piccardo P; Dlouhy SR; Lievens PM; Young K; Bird TD; Nochlin D; Dickson DW; Vinters HV; Zimmerman TR; Mackenzie IR; Kish SJ; Ang LC; De Carli C; Pocchiari M; Brown P; Gibbs CJ; Gajdusek DC; Bugiani O; Ironside J; Tagliavini F; Ghetti B
J Neuropathol Exp Neurol; 1998 Oct; 57(10):979-88. PubMed ID: 9786248
[TBL] [Abstract][Full Text] [Related]
19. Gerstmann-Sträussler-Scheinker Disease with F198S Mutation Induces Independent Tau and Prion Protein Pathologies in Bank Voles.
Bruno R; Pirisinu L; Riccardi G; D'Agostino C; De Cecco E; Legname G; Cardone F; Gambetti P; Nonno R; Agrimi U; Di Bari MA
Biomolecules; 2022 Oct; 12(10):. PubMed ID: 36291746
[TBL] [Abstract][Full Text] [Related]
20. Description of the first Spanish case of Gerstmann-Sträussler-Scheinker disease with A117V variant: clinical, histopathological and biochemical characterization.
Eraña H; San Millán B; Díaz-Domínguez CM; Charco JM; Rodríguez R; Viéitez I; Pereda A; Yañez R; Geijo M; Navarro C; Perez de Nanclares G; Teijeira S; Castilla J
J Neurol; 2022 Aug; 269(8):4253-4263. PubMed ID: 35294616
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
