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2. 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]
3. 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]
4. 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]
5. 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]
6. 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]
7. Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Sträussler-Scheinker disease. Parchi P; Chen SG; Brown P; Zou W; Capellari S; Budka H; Hainfellner J; Reyes PF; Golden GT; Hauw JJ; Gajdusek DC; Gambetti P Proc Natl Acad Sci U S A; 1998 Jul; 95(14):8322-7. PubMed ID: 9653185 [TBL] [Abstract][Full Text] [Related]
8. The epsilon isoform of 14-3-3 protein is a component of the prion protein amyloid deposits of Gerstmann-Sträussler-Scheinker disease. Di Fede G; Giaccone G; Limido L; Mangieri M; Suardi S; Puoti G; Morbin M; Mazzoleni G; Ghetti B; Tagliavini F J Neuropathol Exp Neurol; 2007 Feb; 66(2):124-30. PubMed ID: 17278997 [TBL] [Abstract][Full Text] [Related]
9. 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]
10. 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]
11. 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]
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. 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]
14. Gerstmann-Sträussler-Scheinker disease (PRNP P102L): amyloid deposits are best recognized by antibodies directed to epitopes in PrP region 90-165. Piccardo P; Ghetti B; Dickson DW; Vinters HV; Giaccone G; Bugiani O; Tagliavini F; Young K; Dlouhy SR; Seiler C J Neuropathol Exp Neurol; 1995 Nov; 54(6):790-801. PubMed ID: 7595652 [TBL] [Abstract][Full Text] [Related]
15. A novel PRNP Y218N mutation in Gerstmann-Sträussler-Scheinker disease with neurofibrillary degeneration. Alzualde A; Indakoetxea B; Ferrer I; Moreno F; Barandiaran M; Gorostidi A; Estanga A; Ruiz I; Calero M; van Leeuwen FW; Atares B; Juste R; Rodriguez-Martínez AB; López de Munain A J Neuropathol Exp Neurol; 2010 Aug; 69(8):789-800. PubMed ID: 20613639 [TBL] [Abstract][Full Text] [Related]
16. Detection of tau in Gerstmann-Sträussler-Scheinker disease (PRNP F198S) by [ Risacher SL; Farlow MR; Bateman DR; Epperson F; Tallman EF; Richardson R; Murrell JR; Unverzagt FW; Apostolova LG; Bonnin JM; Ghetti B; Saykin AJ Acta Neuropathol Commun; 2018 Oct; 6(1):114. PubMed ID: 30373672 [TBL] [Abstract][Full Text] [Related]