299 related articles for article (PubMed ID: 19675240)
1. 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]
2. 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]
3. 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]
4. 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]
5. 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]
6. 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]
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. Rapamycin delays disease onset and prevents PrP plaque deposition in a mouse model of Gerstmann-Sträussler-Scheinker disease.
Cortes CJ; Qin K; Cook J; Solanki A; Mastrianni JA
J Neurosci; 2012 Sep; 32(36):12396-405. PubMed ID: 22956830
[TBL] [Abstract][Full Text] [Related]
9. 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]
10. 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]
11. 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]
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. Gerstmann-Sträussler-Scheinker syndrome with variable phenotype in a new kindred with PRNP-P102L mutation.
Riudavets MA; Sraka MA; Schultz M; Rojas E; Martinetto H; Begué C; Noher de Halac I; Poleggi A; Equestre M; Pocchiari M; Sevlever G; Taratuto AL
Brain Pathol; 2014 Mar; 24(2):142-7. PubMed ID: 23944754
[TBL] [Abstract][Full Text] [Related]
14. "Dual Disease" TgAD/GSS mice exhibit enhanced Alzheimer's disease pathology and reveal PrP
Qin K; Zhao L; Gregory C; Solanki A; Mastrianni JA
Sci Rep; 2019 Jun; 9(1):8524. PubMed ID: 31189938
[TBL] [Abstract][Full Text] [Related]
15. 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]
16. Gerstmann-Sträussler-Scheinker disease. I. Human diseases.
Liberski PP; Budka H
Folia Neuropathol; 2004; 42 Suppl B():120-40. PubMed ID: 16903147
[TBL] [Abstract][Full Text] [Related]
17. Transgenic mice recapitulate the phenotypic heterogeneity of genetic prion diseases without developing prion infectivity: Role of intracellular PrP retention in neurotoxicity.
Chiesa R; Restelli E; Comerio L; Del Gallo F; Imeri L
Prion; 2016 Mar; 10(2):93-102. PubMed ID: 26864450
[TBL] [Abstract][Full Text] [Related]
18. A Chinese patient of P102L Gerstmann-Sträussler-Scheinker disease contains three other disease-associated mutations in SYNE1.
Wang J; Xiao K; Zhou W; Gao C; Chen C; Shi Q; Dong XP
Prion; 2018 Mar; 12(2):150-155. PubMed ID: 29509064
[TBL] [Abstract][Full Text] [Related]
19. A previously undiagnosed case of Gerstmann-Sträussler-Scheinker disease revealed by PRNP gene analysis in patients with adult-onset ataxia.
Cagnoli C; Brussino A; Sbaiz L; Di Gregorio E; Atzori C; Caroppo P; Orsi L; Migone N; Buffa C; Imperiale D; Brusco A
Mov Disord; 2008 Jul; 23(10):1468-71. PubMed ID: 18566986
[TBL] [Abstract][Full Text] [Related]
20. A 7-kDa prion protein (PrP) fragment, an integral component of the PrP region required for infectivity, is the major amyloid protein in Gerstmann-Sträussler-Scheinker disease A117V.
Tagliavini F; Lievens PM; Tranchant C; Warter JM; Mohr M; Giaccone G; Perini F; Rossi G; Salmona M; Piccardo P; Ghetti B; Beavis RC; Bugiani O; Frangione B; Prelli F
J Biol Chem; 2001 Feb; 276(8):6009-15. PubMed ID: 11087738
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
