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1 xpressing PrP linked to Gerstmann-Straussler Scheinker syndrome, and the failure of gene-targeted mic
5 lly associated with the Gerstmann-Straussler-Scheinker (GSS) phenotype, also shows marked clinical an
8 on disease [also termed Gerstmann-Straussler-Scheinker (GSS) syndrome] with unusual features such as
11 ogical phenotype of the Gerstmann-Straussler-Scheinker disease (GSS) variant linked to the codon 102
12 g(PrP-A116V) mice model Gerstmann-Straussler-Scheinker disease (GSS), a genetic prion disease charact
13 ations corresponding to Gerstmann-Straussler-Scheinker disease (P102L), Creutzfeld-Jakob disease (E20
14 al profile of a case of Gerstmann-Straussler-Scheinker disease associated with a novel prion protein
15 athologically confirmed Gerstmann-Straussler-Scheinker disease displaying a somewhat unusual constell
18 veloped a cell model of Gerstmann-Straussler-Scheinker disease, a neurodegenerative condition charact
19 or was observed for the Gerstmann-Straussler-Scheinker disease-associated F198S mutant, in which case
24 Jakob disease (CJD) and Gerstmann-Straussler-Scheinker F198S disease brains all have an intact C1 cle
25 loss, by expressing the Gerstmann-Straussler-Scheinker haplotype Q217R-129V in human neuroblastoma ce
29 l insomnia (FFI) n = 9, Gerstmann-Straussler-Scheinker syndrome (GSS)) n = 4), patients with Alzheime
30 utzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome (GSS), and fatal familial insomnia.
32 e genetic prion disease Gerstmann-Straussler-Scheinker syndrome can arise from point mutations of pro
33 Jakob disease (CJD) and Gerstmann-Straussler-Scheinker syndrome have been attributed to particular in
34 utations causing CJD or Gerstmann-Straussler-Scheinker syndrome, 6 had positive and 2 had negative RT
35 present with classical Gerstmann-Straussler-Scheinker syndrome, a slowly progressive cerebellar atax
36 dt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia (FFI) wh
37 116V is associated with Gerstmann-Straussler-Scheinker syndrome, but no accumulation of PrP(Sc) is de
39 ase P102L, historically Gerstmann-Straussler-Scheinker syndrome, originates from central England, wit
42 s mutation results in a Gerstmann-Straussler-Scheinker-like disease with extensive PrP amyloid deposi
43 riant associated with a Gerstmann-Straussler-Scheinker-like prion disease) spontaneously forms amyloi
45 ic residues such as the Gerstmann-Straussler-Scheinker-linked leucines can promote the in vitro forma
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