462 related articles for article (PubMed ID: 24398683)
1. 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]
2. 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]
3. 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]
4. 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]
5. Spontaneous generation of anchorless prions in transgenic mice.
Stöhr J; Watts JC; Legname G; Oehler A; Lemus A; Nguyen HO; Sussman J; Wille H; DeArmond SJ; Prusiner SB; Giles K
Proc Natl Acad Sci U S A; 2011 Dec; 108(52):21223-8. PubMed ID: 22160704
[TBL] [Abstract][Full Text] [Related]
6. Novel method for classification of prion diseases by detecting PrP
Koyama S; Yagita K; Hamasaki H; Noguchi H; Shijo M; Matsuzono K; Takase KI; Kai K; Aishima SI; Itoh K; Ninomiya T; Sasagasako N; Honda H
Prion; 2024 Dec; 18(1):40-53. PubMed ID: 38627365
[TBL] [Abstract][Full Text] [Related]
7. Proteinase-K-resistant prion protein isoforms in Gerstmann-Sträussler-Scheinker disease (Indiana kindred).
Piccardo P; Seiler C; Dlouhy SR; Young K; Farlow MR; Prelli F; Frangione B; Bugiani O; Tagliavini F; Ghetti B
J Neuropathol Exp Neurol; 1996 Nov; 55(11):1157-63. PubMed ID: 8939199
[TBL] [Abstract][Full Text] [Related]
8. 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]
9. Silence of resident microglia in GPI anchorless prion disease and activation of microglia in Gerstmann-Sträussler-Scheinker disease and sporadic Creutzfeldt-Jakob disease.
Noguchi H; Koyama S; Yagita K; Shijo M; Matsuzono K; Hamasaki H; Kanemaru T; Okamoto T; Kai K; Aishima S; Abe K; Sasagasako N; Honda H
J Neuropathol Exp Neurol; 2022 Dec; 82(1):38-48. PubMed ID: 36331509
[TBL] [Abstract][Full Text] [Related]
10. 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]
11. Epitope scanning indicates structural differences in brain-derived monomeric and aggregated mutant prion proteins related to genetic prion diseases.
Tapella L; Stravalaci M; Bastone A; Biasini E; Gobbi M; Chiesa R
Biochem J; 2013 Sep; 454(3):417-25. PubMed ID: 23808898
[TBL] [Abstract][Full Text] [Related]
12. Genetic and infectious prion diseases.
Prusiner SB
Arch Neurol; 1993 Nov; 50(11):1129-53. PubMed ID: 8105771
[TBL] [Abstract][Full Text] [Related]
13. 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]
14. A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann-Sträussler syndrome.
Kitamoto T; Yamaguchi K; Doh-ura K; Tateishi J
Neurology; 1991 Feb; 41(2 ( Pt 1)):306-10. PubMed ID: 1671530
[TBL] [Abstract][Full Text] [Related]
15. 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]
16. 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]
17. 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]
18. Molecular biology of prions causing infectious and genetic encephalopathies of humans as well as scrapie of sheep and BSE of cattle.
Prusiner SB
Dev Biol Stand; 1991; 75():55-74. PubMed ID: 1686599
[TBL] [Abstract][Full Text] [Related]
19. Prion protein amyloidosis.
Ghetti B; Piccardo P; Frangione B; Bugiani O; Giaccone G; Young K; Prelli F; Farlow MR; Dlouhy SR; Tagliavini F
Brain Pathol; 1996 Apr; 6(2):127-45. PubMed ID: 8737929
[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]
