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TY  - JOUR
AU  - Prusiner, S.B.
TI  - Inherited prion diseases
JO  - Proc. Natl. Acad. Sci. USA
PY  - 1994///
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TY  - JOUR
AU  - Masters, C.L.
AU  - Gajdusek, D.C.
AU  - Gibbs, C.J.
TI  - Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Straussler syndrome
JO  - Brain
PY  - 1981///
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TY  - JOUR
AU  - Hsiao, K.
TI  - Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome
JO  - Nature
PY  - 1989///
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TY  - JOUR
AU  - Goldfarb, L.G.
TI  - Fatal familial insomnia and familial Creutzfeldt-Jakob disease: Disease phenotype determined by a DNA polymorphism
JO  - Science
PY  - 1992///
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AU  - Kahana, E.
AU  - Milton, A.
AU  - Braham, J.
AU  - Sofer, D.
TI  - Creutzfeldt[ndash]Jakob disease: Focus among Libyan Jews in Israel
JO  - Science
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AU  - Korczyn, A.
AU  - Brown, P.
AU  - Chapman, J.
AU  - Gajdusek, D.C.
TI  - Mutation in codon 200 of scrapie amyloid precursor gene linked to Creutzfeldt-Jakob disease in Sephardicjews of Libyan and non-Libyan origin
JO  - Lancet
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TY  - JOUR
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TI  - Mutationof the prion proteininLibyan Jews with Creutzfeldt-Jakob disease
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TI  - Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease
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AU  - Mitrova, E.
AU  - Brown, P.
AU  - Toh, B.H.
AU  - Gajdusek, D.C.
TI  - Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia
JO  - Lancet
PY  - 1990///
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TY  - JOUR
AU  - Brown, P.
TI  - Familial Creutzfeldt-Jakob disease in Chile is associated with the codon 200 mutation of the PRNP amyloid precursor gene on chromosome 20
JO  - J. Neural. Sci.
PY  - 1992///
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TI  - Inherited prion disease (PrP lysine 200) in Britain: Two case reports
JO  - Br. Med. J.
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AU  - Bertoni, J.M.
AU  - Brown, P.
AU  - Goldfarb, L.
AU  - Gajdusek, D.
AU  - Omaha, N.E.
TI  - Creutzfeldt[ndash]Jakob disease with the PRNP codon 200lys mutation and supranuclear palsy but without myoclonus or periodic EEG complexes (Abstr)
JO  - Neurology
PY  - 1992///
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TY  - JOUR
AU  - Chapman, J.
AU  - Ben-Israel, J.
AU  - Goldhammer, Y.
AU  - Korczyn, A.D.
TI  - The risk of developing Creutzfeldt[ndash]Jakob disease in subjects with the PRNP gene codon 200 point mutation
JO  - Neurology
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TI  - Complete penetrance of Creutzfeldt-Jakob disease in Libyan Jews carrying the E200K mutation in the prion protein gene
JO  - Mol. Med.
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TY  - JOUR
AU  - Prusiner, S.B.
TI  - Molecular biology of prion diseases
JO  - Science
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TI  - Conversion of a-helices into b-sheets features in the formation of the scrapie prion proteins
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TY  - JOUR
AU  - Huang, Z.
TI  - Proposed three-dimensional structure for the cellular prion protein
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AU  - Roller, P.P.
AU  - Gajdusek, D.C.
AU  - Gibbs, C.J.
TI  - Conformational transitions, dissociation, and unfolding of scrapie amyloid (prion) protein
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AU  - Yamaguchi, K.
AU  - Doh-ura, K.
AU  - Tateishi, J.
TI  - A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann-Straussler syndrome
JO  - Neurology
PY  - 1991///
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AU  - Iizuka, R.
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TI  - An amber mutation of prion protein in Gerstmann-Straussler syndrome with mutant PrP plaques
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TY  - JOUR
AU  - Tagliavini, F.
TI  - Amyloid fibrils in Gerstmann-Straussler-Scheinker disease (Indiana and Swedish kindreds) express only PrP peptides encoded by the mutant allele
JO  - Cell
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TI  - Separation and properties of cellular and scrapie prion proteins
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AU  - Groth, D.
AU  - Prusiner, S.B.
TI  - Intracellular accumulation of the cellular prion protein after mutagenesis of its Asn-linked gly-cosylation sites
JO  - Glycobiology
PY  - 1990///
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AU  - Goldfarb, L.G.
TI  - New mutation in scrapie amyloid precursor gene (at codon 178) in Finnish Creutzfeldt[ndash]Jakob kindred
JO  - Lancet
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AU  - Prusiner, S.B.
TI  - Perturbation of the secondary structure of the scrapie prion protein under conditions associated with changes in infectivity
JO  - Proc. Natl. Acad. Sci. USA
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TI  - Synthetic peptides corresponding to different mutated regions of the amyloid gene in familial Creutzfeldt[ndash]Jakob disease show enhanced in vitro formation of morphologically different amyloid fibrils
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TI  - Predicted [alpha]-helical regions of the prion protein when synthesized as peptides form amyloid
JO  - Proc. Natl. Acad. Sci. USA
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AU  - Fraser, P.E.
AU  - Lansbury, P.T.
TI  - A kinetic model for amyloid formation in the prion diseases: Importance of seeding
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AU  - Forloni, G.
TI  - Neurotoxicity of a prion protein fragment
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AU  - Prusiner, S.B.
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AU  - Meiner, Z.
AU  - Halimi, M.
AU  - Polakiewicz, R.D.
AU  - Prusiner, S.B.
AU  - Gabizon, R.
TI  - Presence of the prion protein in peripheral tissues of Libyan Jews with Creutzfeldt[ndash]Jakob disease
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TI  - Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication
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TI  - Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein
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