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.
4. 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]
5. A comparative study of abnormal prion protein isoforms between Gerstmann-Sträussler-Scheinker syndrome and Creutzfeldt-Jakob disease. Furukawa H; Doh-ura K; Kikuchi H; Tateishi J; Iwaki T J Neurol Sci; 1998 Jun; 158(1):71-5. PubMed ID: 9667781 [TBL] [Abstract][Full Text] [Related]
6. 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]
7. 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]
8. Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles. Hsiao K; Dlouhy SR; Farlow MR; Cass C; Da Costa M; Conneally PM; Hodes ME; Ghetti B; Prusiner SB Nat Genet; 1992 Apr; 1(1):68-71. PubMed ID: 1363810 [TBL] [Abstract][Full Text] [Related]
9. 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]
10. Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred. Giaccone G; Verga L; Bugiani O; Frangione B; Serban D; Prusiner SB; Farlow MR; Ghetti B; Tagliavini F Proc Natl Acad Sci U S A; 1992 Oct; 89(19):9349-53. PubMed ID: 1357663 [TBL] [Abstract][Full Text] [Related]
11. Characterization of Anchorless Human PrP With Q227X Stop Mutation Linked to Gerstmann-Sträussler-Scheinker Syndrome In Vivo and In Vitro. Shen P; Dang J; Wang Z; Zhang W; Yuan J; Lang Y; Ding M; Mitchell M; Kong Q; Feng J; Rozemuller AJM; Cui L; Petersen RB; Zou WQ Mol Neurobiol; 2021 Jan; 58(1):21-33. PubMed ID: 32889654 [TBL] [Abstract][Full Text] [Related]
12. Amyloid fibrils in Gerstmann-Sträussler-Scheinker disease (Indiana and Swedish kindreds) express only PrP peptides encoded by the mutant allele. Tagliavini F; Prelli F; Porro M; Rossi G; Giaccone G; Farlow MR; Dlouhy SR; Ghetti B; Bugiani O; Frangione B Cell; 1994 Nov; 79(4):695-703. PubMed ID: 7954833 [TBL] [Abstract][Full Text] [Related]
13. 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]
14. Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene. Dlouhy SR; Hsiao K; Farlow MR; Foroud T; Conneally PM; Johnson P; Prusiner SB; Hodes ME; Ghetti B Nat Genet; 1992 Apr; 1(1):64-7. PubMed ID: 1363809 [TBL] [Abstract][Full Text] [Related]
15. 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]
16. Hereditary prion protein amyloidoses. Ghetti B; Tagliavini F; Takao M; Bugiani O; Piccardo P Clin Lab Med; 2003 Mar; 23(1):65-85, viii. PubMed ID: 12733425 [TBL] [Abstract][Full Text] [Related]
17. Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58. Tagliavini F; Prelli F; Ghiso J; Bugiani O; Serban D; Prusiner SB; Farlow MR; Ghetti B; Frangione B EMBO J; 1991 Mar; 10(3):513-9. PubMed ID: 1672107 [TBL] [Abstract][Full Text] [Related]
18. 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]