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Volumn 11, Issue 2, 1998, Pages 78-97

The prion diseases: Creutzfeldt-Jakob, Gerstmann-Sträussler-Scheinker, and related disorders

Author keywords

[No Author keywords available]

Indexed keywords

HEPARAN; PENTOSAN POLYSULFATE; PRION PROTEIN; SURAMIN;

EID: 0032420150     PISSN: 08919887     EISSN: None     Source Type: Journal    
DOI: 10.1177/089198879801100206     Document Type: Article
Times cited : (14)

References (210)
  • 1
    • 0000049491 scopus 로고
    • Experimental transmission of trembling to the goat
    • Cuillé J, Chelle PL. Experimental transmission of trembling to the goat. C R Seances Acad Sci 1939; 208:1058-1060.
    • (1939) C R Seances Acad Sci , vol.208 , pp. 1058-1060
    • Cuillé, J.1    Chelle, P.L.2
  • 5
    • 49749206702 scopus 로고
    • Encephalopathy in mice produced by inoculation with scrapie brain material
    • Chandler RL. Encephalopathy in mice produced by inoculation with scrapie brain material. Lancet 1961; 1:1378-1379.
    • (1961) Lancet , vol.1 , pp. 1378-1379
    • Chandler, R.L.1
  • 8
    • 0020321767 scopus 로고
    • Novel proteinaceous infectious particles cause scrapie
    • Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science 1982; 216:136-144.
    • (1982) Science , vol.216 , pp. 136-144
    • Prusiner, S.B.1
  • 9
    • 0021023167 scopus 로고
    • A protease-resistant protein is a structural component of the scrapie prion
    • McKinley MP, Bolton DC, Prusiner SB. A protease-resistant protein is a structural component of the scrapie prion. Cell 1983; 35:57-62.
    • (1983) Cell , vol.35 , pp. 57-62
    • McKinley, M.P.1    Bolton, D.C.2    Prusiner, S.B.3
  • 11
    • 0020490156 scopus 로고
    • Identification of a protein that purifies with the scrapie prion
    • Bolton DC, McKinley MP, Prusiner SB. Identification of a protein that purifies with the scrapie prion. Science 1982; 218:1309-1311.
    • (1982) Science , vol.218 , pp. 1309-1311
    • Bolton, D.C.1    McKinley, M.P.2    Prusiner, S.B.3
  • 12
    • 0022005315 scopus 로고
    • A cellular gene encodes scrapie PrP 27-30 protein
    • Oesch B, Westaway D, Wälchli M, et al. A cellular gene encodes scrapie PrP 27-30 protein. Cell 1985; 40:735-746.
    • (1985) Cell , vol.40 , pp. 735-746
    • Oesch, B.1    Westaway, D.2    Wälchli, M.3
  • 13
    • 0027182522 scopus 로고
    • Conformational transitions, dissociation, and unfolding of scrapie amyloid (prion) protein
    • Safar J, Roller PP, Gajdusek DC, Gibbs CJ, Conformational transitions, dissociation, and unfolding of scrapie amyloid (prion) protein. JBiol Chem 1993; 268:20276-20284.
    • (1993) JBiol Chem , vol.268 , pp. 20276-20284
    • Safar, J.1    Roller, P.P.2    Gajdusek, D.C.3    Gibbs, C.J.4    Jr5
  • 14
    • 0026600865 scopus 로고
    • Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein
    • Büeler H, Fischer M, Lang Y, et al. Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature 1992; 356:577-582.
    • (1992) Nature , vol.356 , pp. 577-582
    • Büeler, H.1    Fischer, M.2    Lang, Y.3
  • 15
    • 0027319326 scopus 로고
    • Mice devoid of PrP are resistant to scrapie
    • Büeler H, Aguzzi A, Sailer A, et al. Mice devoid of PrP are resistant to scrapie. Cell 1993; 73:1339-1347.
    • (1993) Cell , vol.73 , pp. 1339-1347
    • Büeler, H.1    Aguzzi, A.2    Sailer, A.3
  • 16
    • 0028343093 scopus 로고
    • No propagation of prions in mice devoid of PrP
    • Sailer A, Büeler H, Fischer M, et al. No propagation of prions in mice devoid of PrP. Cell 1994; 77:967-968.
    • (1994) Cell , vol.77 , pp. 967-968
    • Sailer, A.1    Büeler, H.2    Fischer, M.3
  • 17
    • 0028882424 scopus 로고
    • Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein
    • Telling GC, Scott M, Mastrianni J, et al. Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell 1995; 83:79-90.
    • (1995) Cell , vol.83 , pp. 79-90
    • Telling, G.C.1    Scott, M.2    Mastrianni, J.3
  • 18
    • 0030967895 scopus 로고    scopus 로고
    • Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform
    • James TL, Liu H, Ulyanov NB, et al. Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform. Proc Natl Acad Sci USA 1997; 94:10086-10091.
    • (1997) Proc Natl Acad Sci USA , vol.94 , pp. 10086-10091
    • James, T.L.1    Liu, H.2    Ulyanov, N.B.3
  • 19
    • 0030931519 scopus 로고    scopus 로고
    • Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation
    • Kaneko K, Zulianello L, Scott M, et al. Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation. Proc Natl Acad Sci USA 1997; 94:10069-10074.
    • (1997) Proc Natl Acad Sci USA , vol.94 , pp. 10069-10074
    • Kaneko, K.1    Zulianello, L.2    Scott, M.3
  • 20
    • 0023052247 scopus 로고
    • Human prion protein cDNA: Molecular cloning, chromosomal mapping, and biological implication
    • Liao Y-C, Lebo RV, Clawson GA, Smuckler EA. Human prion protein cDNA: molecular cloning, chromosomal mapping, and biological implication. Science 1986; 233:364-367.
    • (1986) Science , vol.233 , pp. 364-367
    • Liao, Y.-C.1    Lebo, R.V.2    Clawson, G.A.3    Smuckler, E.A.4
  • 21
    • 0023663071 scopus 로고
    • Scrapie prion protein contains a phosphatidylinositol glycolipid
    • Stahl N, Borchelt DR, Hsiao K, Prusiner SB. Scrapie prion protein contains a phosphatidylinositol glycolipid. Cell 1987; 51:229-240.
    • (1987) Cell , vol.51 , pp. 229-240
    • Stahl, N.1    Borchelt, D.R.2    Hsiao, K.3    Prusiner, S.B.4
  • 22
    • 0026069894 scopus 로고
    • Proteinase-resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity
    • Jendroska K, Heinzel FP, Tbrchia M, et al. Proteinase-resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity. Neurology 1991; 41:1482-1490.
    • (1991) Neurology , vol.41 , pp. 1482-1490
    • Jendroska, K.1    Heinzel, F.P.2    Tbrchia, M.3
  • 24
    • 0026567814 scopus 로고
    • Nearly ubiquitous tissue distribution of the scrapie agent precursor protein
    • Bendheim PE, Brown HR, Rudelli RD, et al. Nearly ubiquitous tissue distribution of the scrapie agent precursor protein. Neurology 1992; 42:149-156.
    • (1992) Neurology , vol.42 , pp. 149-156
    • Bendheim, P.E.1    Brown, H.R.2    Rudelli, R.D.3
  • 25
    • 0030601023 scopus 로고    scopus 로고
    • PrP2MO is a normal soluble prion protein fragment released by human platelets
    • Perini F, Vidai R, Ghetti B, et al. PrP2MO is a normal soluble prion protein fragment released by human platelets. Biochem Biophys Res Commun 1996; 223:572-577.
    • (1996) Biochem Biophys Res Commun , vol.223 , pp. 572-577
    • Perini, F.1    Vidai, R.2    Ghetti, B.3
  • 26
    • 0028356488 scopus 로고
    • Rapid anterograde axonal transport of the cellular prion glycoprotein in the peripheral and central nervous systems
    • Borchelt DR, Koliatsis VE, Guarnieri M, et al. Rapid anterograde axonal transport of the cellular prion glycoprotein in the peripheral and central nervous systems. J Biol Chem 1994; 269:14711-14714.
    • (1994) J Biol Chem , vol.269 , pp. 14711-14714
    • Borchelt, D.R.1    Koliatsis, V.E.2    Guarnieri, M.3
  • 27
    • 0029061953 scopus 로고
    • Human muscle macrophages express b-amyloid precursor and prion proteins and their mRNAs
    • Askanas V, Sarkozi E, Bilak M, et al. Human muscle macrophages express b-amyloid precursor and prion proteins and their mRNAs. Neuroreport 1995; 6:1045-1049.
    • (1995) Neuroreport , vol.6 , pp. 1045-1049
    • Askanas, V.1    Sarkozi, E.2    Bilak, M.3
  • 28
    • 0028052363 scopus 로고
    • Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins
    • Westaway D, DeArmond SJ, Cayetano-Canlas J, et al. Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins. Cell 1994; 76:117-129.
    • (1994) Cell , vol.76 , pp. 117-129
    • Westaway, D.1    Dearmond, S.J.2    Cayetano-Canlas, J.3
  • 29
    • 0027997387 scopus 로고
    • Prion protein is necessary for normal synaptic function
    • Collinge J, Whittington MA, Sidle KG, et al. Prion protein is necessary for normal synaptic function. Nature 1994; 370:295-297.
    • (1994) Nature , vol.370 , pp. 295-297
    • Collinge, J.1    Whittington, M.A.2    Sidle, K.G.3
  • 30
    • 0028802150 scopus 로고
    • Rescue of neurophysiological phenotype seen in PrP null mice by transgene encoding human prion protein
    • Whittington MA, Sidle KCL, Gowland I, et al. Rescue of neurophysiological phenotype seen in PrP null mice by transgene encoding human prion protein. Nat Genet 1995; 9:197-201.
    • (1995) Nat Genet , vol.9 , pp. 197-201
    • Whittington, M.A.1    Sidle, K.C.L.2    Gowland, I.3
  • 31
    • 0029265461 scopus 로고
    • PrP gene dosage and long term potentiation [letter]
    • Manson JC, Hope J, Clarke AR, et al. PrP gene dosage and long term potentiation [letter]. Neurodegeneration 1995; 4:113-114.
    • (1995) Neurodegeneration , vol.4 , pp. 113-114
    • Manson, J.C.1    Hope, J.2    Clarke, A.R.3
  • 32
    • 0031444294 scopus 로고    scopus 로고
    • The cellular prion protein binds copper in vivo
    • Brown DR, Qin K, Herms JW, et al. The cellular prion protein binds copper in vivo. Nature 1997; 390:684-687.
    • (1997) Nature , vol.390 , pp. 684-687
    • Brown, D.R.1    Qin, K.2    Herms, J.W.3
  • 33
    • 0037965529 scopus 로고    scopus 로고
    • Prion protein selectively binds copper-II-ions
    • Stöckel J, Safar J, Wallace AC, et al. Prion protein selectively binds copper-II-ions. Biochemistry 1998; 37:7185-7193.
    • (1998) Biochemistry , vol.37 , pp. 7185-7193
    • Stöckel, J.1    Safar, J.2    Wallace, A.C.3
  • 34
    • 0029916617 scopus 로고    scopus 로고
    • Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus
    • Lledo P-M, Tremblay P, DeArmond SJ, et al. Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus. Proc Natl Acad Sci USA 1996; 93:2403-2407.
    • (1996) Proc Natl Acad Sci USA , vol.93 , pp. 2403-2407
    • Lledo, P.-M.1    Tremblay, P.2    Dearmond, S.J.3
  • 35
    • 15844421385 scopus 로고    scopus 로고
    • Altered circadian activity rhythms and sleep in mice devoid of prion protein
    • Ibbler I, Gaus SE, Deboer T, et al. Altered circadian activity rhythms and sleep in mice devoid of prion protein. Nature 1996; 380:639-642.
    • (1996) Nature , vol.380 , pp. 639-642
    • Ibbler, I.1    Gaus, S.E.2    Deboer, T.3
  • 36
    • 0023243707 scopus 로고
    • The epidemiology of Creutzfeldt-Jakob disease: Conclusion of 15-year investigation in France and review of the world literature
    • Brown P, Cathala F, Raubertas RF, et al. The epidemiology of Creutzfeldt-Jakob disease: conclusion of 15-year investigation in France and review of the world literature. Neurology 1987; 37:895-904.
    • (1987) Neurology , vol.37 , pp. 895-904
    • Brown, P.1    Cathala, F.2    Raubertas, R.F.3
  • 37
    • 0028981199 scopus 로고
    • Complete penetrance of Creutzfeldt-Jakob disease in Libyan Jews carrying the E200K mutation in the prion protein gene
    • Spudich S, Mastrianni JA, Wrensch M, et al. Complete penetrance of Creutzfeldt-Jakob disease in Libyan Jews carrying the E200K mutation in the prion protein gene. Mol Med 1995; 1:607-613.
    • (1995) Mol Med , vol.1 , pp. 607-613
    • Spudich, S.1    Mastrianni, J.A.2    Wrensch, M.3
  • 38
    • 0024519771 scopus 로고
    • Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome
    • Hsiao K, Baker HF, Crow TJ, et al. Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome. Nature 1989; 338:342-345.
    • (1989) Nature , vol.338 , pp. 342-345
    • Hsiao, K.1    Baker, H.F.2    Crow, T.J.3
  • 39
    • 0025013882 scopus 로고
    • Neurofibrillary tangles of the Indiana kindred of Gerstmann-Straussler-Scheinker disease share antigenic determinants with those of Alzheimer disease
    • Giaccone G, Tagliavini F, Verga L, et al. Neurofibrillary tangles of the Indiana kindred of Gerstmann-Straussler-Scheinker disease share antigenic determinants with those of Alzheimer disease. Brain Res 1990; 530:325-329.
    • (1990) Brain Res , vol.530 , pp. 325-329
    • Giaccone, G.1    Tagliavini, F.2    Verga, L.3
  • 40
    • 0007856994 scopus 로고
    • Atypical GerstmannStraussler-Scheinker syndrome with neurofibrillary tangles: No mutation in the prion protein open-reading-frame in a patient of the Indiana kindred
    • Hsiao K, Cass C, Conneally PM, et al. Atypical GerstmannStraussler-Scheinker syndrome with neurofibrillary tangles: no mutation in the prion protein open-reading-frame in a patient of the Indiana kindred. Neurobiol Aging 1990; 11:302.
    • (1990) Neurobiol Aging , vol.11 , pp. 302
    • Hsiao, K.1    Cass, C.2    Conneally, P.M.3
  • 42
    • 13344295093 scopus 로고    scopus 로고
    • Vascular variant of prion protein cerebral amyloidosis with t-positive neurofibrillary tangles: The phenotype of the stop codon 145 mutation in PRNP
    • Ghetti B, Piccardo P, Spillantini MG, et al. Vascular variant of prion protein cerebral amyloidosis with t-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP. Proc Natl Acad Sci USA 1996; 93:744-748.
    • (1996) Proc Natl Acad Sci USA , vol.93 , pp. 744-748
    • Ghetti, B.1    Piccardo, P.2    Spillantini, M.G.3
  • 43
    • 0028043661 scopus 로고
    • Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy
    • Bessen RA, Marsh RF. Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy. J Virol 1994; 68:7859-7868.
    • (1994) J Virol , vol.68 , pp. 7859-7868
    • Bessen, R.A.1    Marsh, R.F.2
  • 44
    • 0026558780 scopus 로고
    • Identification of two biologically distinct strains of transmissible mink encephalopathy in hamsters
    • Bessen RA, Marsh RF. Identification of two biologically distinct strains of transmissible mink encephalopathy in hamsters. J Gen Virol 1992; 73:329-334.
    • (1992) J Gen Virol , vol.73 , pp. 329-334
    • Bessen, R.A.1    Marsh, R.F.2
  • 45
    • 0029831213 scopus 로고    scopus 로고
    • Molecular analysis of prion strain variation and the aetiology of new variant CJD
    • Collinge J, Sidle KCL, Meads J, et al. Molecular analysis of prion strain variation and the aetiology of new variant CJD. Nature 1996; 383:685-690.
    • (1996) Nature , vol.383 , pp. 685-690
    • Collinge, J.1    Sidle, K.C.L.2    Meads, J.3
  • 46
    • 0030775632 scopus 로고    scopus 로고
    • Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent
    • Bruce ME, Will RG, Ironside JW, et al. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 1997; 389:498-501.
    • (1997) Nature , vol.389 , pp. 498-501
    • Bruce, M.E.1    Will, R.G.2    Ironside, J.W.3
  • 48
    • 85044683608 scopus 로고    scopus 로고
    • Biochemical typing of scrapie strains [reply]
    • Collinge J, Hill AF, Sidle KCL, et al. Biochemical typing of scrapie strains [reply]. Nature 1997; 386:564.
    • (1997) Nature , vol.386 , pp. 564
    • Collinge, J.1    Hill, A.F.2    Sidle, K.C.L.3
  • 49
    • 13144275223 scopus 로고    scopus 로고
    • Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice
    • Scott MR, Safar J, Telling G, et al. Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice. Proc Natl Acad SciUSA 1997; 94:14279-14284.
    • (1997) Proc Natl Acad SciUSA , vol.94 , pp. 14279-14284
    • Scott, M.R.1    Safar, J.2    Telling, G.3
  • 50
    • 0344222186 scopus 로고    scopus 로고
    • Selective neuronal targeting in prion disease
    • DeArmond SJ, Sânchez H, Yehiely F, et al. Selective neuronal targeting in prion disease. Neuron 1997; 19:1337-1348.
    • (1997) Neuron , vol.19 , pp. 1337-1348
    • Dearmond, S.J.1    Sânchez, H.2    Yehiely, F.3
  • 51
    • 0021171932 scopus 로고
    • CreutzfeldtJakob disease of long duration: Clinicopathological characteristics, transmissibility, and differential diagnosis
    • Brown P, Rodgers-Johnson P, Cathala F, et al. CreutzfeldtJakob disease of long duration: clinicopathological characteristics, transmissibility, and differential diagnosis. Ann Neurol 1984; 16:295-304.
    • (1984) Ann Neurol , vol.16 , pp. 295-304
    • Brown, P.1    Rodgers-Johnson, P.2    Cathala, F.3
  • 52
    • 0022973492 scopus 로고
    • CreutzfeldtJakob disease: Clinical analysis of a consecutive series of 230 neuropathologically verified cases
    • Brown P, Cathala F, Castaigne P, Gajdusek DC. CreutzfeldtJakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol 1986; 20:597-602.
    • (1986) Ann Neurol , vol.20 , pp. 597-602
    • Brown, P.1    Cathala, F.2    Castaigne, P.3    Gajdusek, D.C.4
  • 53
    • 0018844661 scopus 로고
    • Serial EEC findings in 27 cases of Creutzfeldt-Jakob disease
    • Chiafalo N, Fuentes AN, Galvez S. Serial EEC findings in 27 cases of Creutzfeldt-Jakob disease. Arch Neurol 1980; 37: 143-145.
    • (1980) Arch Neurol , vol.37 , pp. 143-145
    • Chiafalo, N.1    Fuentes, A.N.2    Galvez, S.3
  • 54
    • 0015749015 scopus 로고
    • The ataxic form of Creutzfeldt-Jakob disease
    • Gomori AJ, Partnow MJ, Horoupian DS, et al. The ataxic form of Creutzfeldt-Jakob disease. Arch Neurol 1973; 29: 318-323.
    • (1973) Arch Neurol , vol.29 , pp. 318-323
    • Gomori, A.J.1    Partnow, M.J.2    Horoupian, D.S.3
  • 55
    • 0020450811 scopus 로고
    • The pathogenesis of transmissible spongiform encephalopathy - An ultrastructural study
    • Beck E, Daniel PM, Davey AJ, et al. The pathogenesis of transmissible spongiform encephalopathy - an ultrastructural study. Brain 1982; 105:755-786.
    • (1982) Brain , vol.105 , pp. 755-786
    • Beck, E.1    Daniel, P.M.2    Davey, A.J.3
  • 60
    • 0028102794 scopus 로고
    • Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein
    • Telling GC, Scott M, Hsiao KK, et al. Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. Proc Natl Acad SciUSA 1994; 91:9936-9940.
    • (1994) Proc Natl Acad SciUSA , vol.91 , pp. 9936-9940
    • Telling, G.C.1    Scott, M.2    Hsiao, K.K.3
  • 61
    • 0007867097 scopus 로고
    • Creutzfeldt-Jakob disease following human pituitary-derived growth hormone administration
    • Anderson JR, Allen CMC, Weller RO. Creutzfeldt-Jakob disease following human pituitary-derived growth hormone administration. Br Neuropathol Sac Proc 1990; 16:543.
    • (1990) Br Neuropathol Sac Proc , vol.16 , pp. 543
    • Anderson, J.R.1    Allen, C.M.C.2    Weller, R.O.3
  • 65
    • 0025963199 scopus 로고
    • CreutzfeldtJakob disease in pituitary growth hormone recipients in the United States
    • Fradkin JE, Schonberger LB, Mills JL, et al. CreutzfeldtJakob disease in pituitary growth hormone recipients in the United States. JAMA 1991; 265:880-884.
    • (1991) JAMA , vol.265 , pp. 880-884
    • Fradkin, J.E.1    Schonberger, L.B.2    Mills, J.L.3
  • 67
    • 0026667331 scopus 로고
    • Friendly fire in medicine: Hormones, homografts, and Creutzfeldt-Jakob disease
    • Brown P, Preece MA, Will RG. Friendly fire in medicine: hormones, homografts, and Creutzfeldt-Jakob disease. Lancet 1992; 340:24-27.
    • (1992) Lancet , vol.340 , pp. 24-27
    • Brown, P.1    Preece, M.A.2
  • 68
    • 0023373951 scopus 로고
    • Jakob-Creutzfeldt disease associated with cadaveric dura [letter]
    • Otto D. Jakob-Creutzfeldt disease associated with cadaveric dura [letter]. JNeurosurg 1987; 67:149-150.
    • (1987) JNeurosurg , vol.67 , pp. 149-150
    • Otto, D.1
  • 69
    • 0023804449 scopus 로고
    • Creutzfeldt-Jakob disease probably acquired from a cadaveric dura mater graft
    • Thadani V, Penar PL, Partington J, et al. Creutzfeldt-Jakob disease probably acquired from a cadaveric dura mater graft. Case report. JNeurosurg 1988; 69:766-769.
    • (1988) Case Report. JNeurosurg , vol.69 , pp. 766-769
    • Thadani, V.1    Penar, P.L.2    Partington, J.3
  • 70
    • 84941817285 scopus 로고
    • Creutzfeldt-Jakob disease in a second patient who received a cadaveric dura mater graft
    • Nisbet TJ, MacDonaldson I, Bishara SN. Creutzfeldt-Jakob disease in a second patient who received a cadaveric dura mater graft. JAMA 1989; 261:1118.
    • (1989) JAMA , vol.261 , pp. 1118
    • Nisbet, T.J.1    MacDonaldson, I.2    Bishara, S.N.3
  • 71
    • 0024471762 scopus 로고
    • Transmission of Creutzfeldt-Jakob disease by durai cadaveric graft [letter, comment]
    • Masullo C, Pocchiari M, Macchi G, et al. Transmission of Creutzfeldt-Jakob disease by durai cadaveric graft [letter, comment]. JNeurosurg 1989; 71:954-955.
    • (1989) JNeurosurg , vol.71 , pp. 954-955
    • Masullo, C.1    Pocchiari, M.2    Macchi, G.3
  • 73
    • 0025847996 scopus 로고
    • Creutzfeldt-Jakob disease in a patient with a cadaveric durai graft
    • Miyashita K, Inuzuka T, Kondo H, et al. Creutzfeldt-Jakob disease in a patient with a cadaveric durai graft. Neurology 1991; 41:940-941.
    • (1991) Neurology , vol.41 , pp. 940-941
    • Miyashita, K.1    Inuzuka, T.2    Kondo, H.3
  • 74
    • 0032491835 scopus 로고    scopus 로고
    • Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts in Japan, Janu-ary 1979-May 1996 (Reprinted from MMWR, Vol. 46, pp. 1066-1069,1997)
    • Sato T, Hosh K, Yoshino H, et al. Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts in Japan, Janu-ary 1979-May 1996 (Reprinted from MMWR, Vol. 46, pp. 1066-1069,1997). JAMA 1998; 279(N1):11-12.
    • (1998) JAMA , vol.279 , pp. 11-12
    • Sato, T.1    Hosh, K.2    Yoshino, H.3
  • 75
    • 0016398968 scopus 로고
    • Possible person to person transmission of Creutzfeldt-Jakob disease
    • Dufiy P, Wolf J, Collins G, et al. Possible person to person transmission of Creutzfeldt-Jakob disease. N Engl J Med 1974; 290:692-693.
    • (1974) N Engl J Med , vol.290 , pp. 692-693
    • Dufiy, P.1    Wolf, J.2    Collins, G.3
  • 76
    • 0017367439 scopus 로고
    • Danger of accidental person to person transmission of Creutzfeldt-Jakob disease by surgery
    • Bernouilli C, Siegfried J, Baumgartner G, et al. Danger of accidental person to person transmission of Creutzfeldt-Jakob disease by surgery. Lancet 1977; 1:478-479.
    • (1977) Lancet , vol.1 , pp. 478-479
    • Bernouilli, C.1    Siegfried, J.2    Baumgartner, G.3
  • 77
    • 0025221785 scopus 로고
    • Creutzfeldt-Jakob disease in a recipient of human pituitary-derived gonadotrophin
    • Cochius JI, Mack K, Burns RJ, et al. Creutzfeldt-Jakob disease in a recipient of human pituitary-derived gonadotrophin. AustNZJMed 1990; 20:592-593.
    • (1990) AustNZJMed , vol.20 , pp. 592-593
    • Cochius, J.I.1    Mack, K.2    Burns, R.J.3
  • 78
    • 0026464707 scopus 로고
    • Creutzfeldt-Jakob disease in a recipient of human pituitary-derived gonadotrophin: A second case
    • Cochius JI, Hyman N, Esiri MM. Creutzfeldt-Jakob disease in a recipient of human pituitary-derived gonadotrophin: a second case. J Neurol Neurosurg Psychiatry 1992; 55:1094-1095.
    • (1992) J Neurol Neurosurg Psychiatry , vol.55 , pp. 1094-1095
    • Cochius, J.I.1    Hyman, N.2    Esiri, M.M.3
  • 79
    • 0027214250 scopus 로고
    • Creutzfeldt-Jakob disease after pituitary gonadotrophins
    • Healy DL, Evans J. Creutzfeldt-Jakob disease after pituitary gonadotrophins. BMJ 1993; 307:517-518.
    • (1993) BMJ , vol.307 , pp. 517-518
    • Healy, D.L.1    Evans, J.2
  • 80
    • 0028971675 scopus 로고
    • Sporadic Creutzfeldt-Jakob disease in a 18-year-old in the UK [letter]
    • Bateman D, Hilton D, Love S, et al. Sporadic Creutzfeldt-Jakob disease in a 18-year-old in the UK [letter]. Lancet 1995; 346:1155-1156.
    • (1995) Lancet , vol.346 , pp. 1155-1156
    • Bateman, D.1    Hilton, D.2    Love, S.3
  • 81
    • 0028971675 scopus 로고
    • Sporadic CreutzfeldtJakob disease in a 16-year-old in the UK [letter]
    • Britton TC, Al-Sarraj S, Shaw C, et al. Sporadic CreutzfeldtJakob disease in a 16-year-old in the UK [letter]. Lancet 1995; 346:1155.
    • (1995) Lancet , vol.346 , pp. 1155
    • Britton, T.C.1    Al-Sarraj, S.2    Shaw, C.3
  • 82
    • 0344342110 scopus 로고    scopus 로고
    • New variant of Creutzfeldt-Jakob disease in a 26-year-old French man [letter]
    • Chazot G, Broussolle E, Lapras CI, et al. New variant of Creutzfeldt-Jakob disease in a 26-year-old French man [letter]. Lancet 1996; 347:1181.
    • (1996) Lancet , vol.347 , pp. 1181
    • Chazot, G.1    Broussolle, E.2    Lapras, C.I.3
  • 83
    • 0029806963 scopus 로고    scopus 로고
    • CreutzfeldtJakob disease in a 52-year-old woman with florid plaques [letter]
    • Kopp N, Streichenberger N, Deslys JP, et al. CreutzfeldtJakob disease in a 52-year-old woman with florid plaques [letter]. Lancet 1996; 348:1239-1240.
    • (1996) Lancet , vol.348 , pp. 1239-1240
    • Kopp, N.1    Streichenberger, N.2    Deslys, J.P.3
  • 84
    • 0342951746 scopus 로고    scopus 로고
    • A new variant of Creutzfeldt-Jakob disease in the UK
    • Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996; 347: 921-925.
    • (1996) Lancet , vol.347 , pp. 921-925
    • Will, R.G.1    Ironside, J.W.2    Zeidler, M.3
  • 85
    • 0001385519 scopus 로고
    • Über ein noch nicht beschriebenes Reflexphanomen bei einer Erkrankung des zerebellaren Systems
    • Gerstmann J. Über ein noch nicht beschriebenes Reflexphanomen bei einer Erkrankung des zerebellaren Systems. Wien Med Wochenschr 1928; 78:906.
    • (1928) Wien Med Wochenschr , vol.78 , pp. 906
    • Gerstmann, J.1
  • 86
    • 51849178459 scopus 로고
    • Über eine eigenartige hereditär-familiäre Erkrankung des Zentralnervensystems zugleich ein Beitrag zur frage des vorzeitigen lokalen AI terns
    • Gerstmann J, Sträussler E, Scheinker I. Über eine eigenartige hereditär-familiäre Erkrankung des Zentralnervensystems zugleich ein Beitrag zur frage des vorzeitigen lokalen AI terns. Z Neurol 1936; 154:736.
    • (1936) Z Neurol , vol.154 , pp. 736
    • Gerstmann, J.1    Sträussler, E.2    Scheinker, I.3
  • 87
    • 0026651376 scopus 로고
    • The sequential development of abnormal prion protein accumulation in mice with Creutzfeldt-Jakob disease
    • Muramoto T, Kitamoto T, Tateishi J, Goto I. The sequential development of abnormal prion protein accumulation in mice with Creutzfeldt-Jakob disease. Am J Pathol 1992; 140: 1411-1420.
    • (1992) Am J Pathol , vol.140 , pp. 1411-1420
    • Muramoto, T.1    Kitamoto, T.2    Tateishi, J.3    Goto, I.4
  • 89
    • 0026552043 scopus 로고
    • Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene
    • Medori R, Tritschler H-J, LeBIanc A, et al. Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene. N Engl J Med 1992; 326:444-449.
    • (1992) N Engl J Med , vol.326 , pp. 444-449
    • Medori, R.1    Tritschler, H.-J.2    Lebianc, A.3
  • 90
    • 0026690906 scopus 로고
    • Fatal familial insomnia: A second kindred with mutation of prion protein gene at codon 178
    • Medori R, Montagna P, Tritschler HJ, et al. Fatal familial insomnia: a second kindred with mutation of prion protein gene at codon 178. Neurology 1992; 42:669-670.
    • (1992) Neurology , vol.42 , pp. 669-670
    • Medori, R.1    Montagna, P.2    Tritschler, H.J.3
  • 91
    • 0026785544 scopus 로고
    • Analysis of the prion protein gene in thalamic dementia
    • Petersen RB, Tabaton M, Berg L, et al. Analysis of the prion protein gene in thalamic dementia. Neurology 1992; 42: 1859-1863.
    • (1992) Neurology , vol.42 , pp. 1859-1863
    • Petersen, R.B.1    Tabaton, M.2    Berg, L.3
  • 92
    • 0028835989 scopus 로고
    • Fatal familial insomnia and familial Creutzfeldt-Jakob disease: Clinical, pathological and molecular features [review]
    • Gambetti P, Parchi P, Petersen RB, et al. Fatal familial insomnia and familial Creutzfeldt-Jakob disease: clinical, pathological and molecular features [review]. Brain Pathol 1995; 5:43-51.
    • (1995) Brain Pathol , vol.5 , pp. 43-51
    • Gambetti, P.1    Parchi, P.2    Petersen, R.B.3
  • 93
    • 0026801958 scopus 로고
    • A PrP gene codon 178 base substitution and a 24-bp interstitial deletion in familial Creutzfeldt-Jakob disease
    • Bosque PJ, Vnencak-Jones CL, Johnson MD, et al. A PrP gene codon 178 base substitution and a 24-bp interstitial deletion in familial Creutzfeldt-Jakob disease. Neurology 1992; 42:1864-1870.
    • (1992) Neurology , vol.42 , pp. 1864-1870
    • Bosque, P.J.1    Vnencak-Jones, C.L.2    Johnson, M.D.3
  • 94
    • 0029031422 scopus 로고
    • Clinical and genetic studies of fatal familial insomnia
    • Reder AT, Mednick AS, Brown P, et al. Clinical and genetic studies of fatal familial insomnia. Neurology 1995; 45:1068-1075.
    • (1995) Neurology , vol.45 , pp. 1068-1075
    • Reder, A.T.1    Mednick, A.S.2    Brown, P.3
  • 96
    • 0029961971 scopus 로고    scopus 로고
    • Fatal familial insomnia with a mutation at codon 178 of the prion protein gene: First report from Japan
    • Nagayama M, Shinohara Y, Furukawa H, Kitamoto T. Fatal familial insomnia with a mutation at codon 178 of the prion protein gene: first report from Japan. Neurology 1996; 47: 1313-1316.
    • (1996) Neurology , vol.47 , pp. 1313-1316
    • Nagayama, M.1    Shinohara, Y.2    Furukawa, H.3    Kitamoto, T.4
  • 97
    • 0027787024 scopus 로고
    • FFDG PET in fatal familial insomnia: The functional effects of thalamic lesions
    • 18 F]FDG PET in fatal familial insomnia: the functional effects of thalamic lesions. Neurology 1993; 43:2565-2569. j
    • (1993) Neurology , vol.43 , pp. 2565-2569
    • Perani, D.1    Cortelli, P.2    Lucignani, G.3
  • 99
    • 0026563279 scopus 로고
    • Fatal familial insomnia: Clinical and pathological study of five new cases
    • Manetto V, Medori R, Cortelli P, et al. Fatal familial insomnia: clinical and pathological study of five new cases. Neurology 1992; 42:312-319.
    • (1992) Neurology , vol.42 , pp. 312-319
    • Manetto, V.1    Medori, R.2    Cortelli, P.3
  • 101
    • 0030821246 scopus 로고    scopus 로고
    • The D178N (cis 129M) fatal familial insomnia mutation associated with diverse clinicopathologic phenotypes in an Australian kindred
    • McLean CA, Storey E, Gardner RJM, et al. The D178N (cis 129M) fatal familial insomnia mutation associated with diverse clinicopathologic phenotypes in an Australian kindred. Neurology 1997; 49:552-558.
    • (1997) Neurology , vol.49 , pp. 552-558
    • McLean, C.A.1    Storey, E.2    Gardner, R.J.M.3
  • 102
    • 0029042656 scopus 로고
    • Regional distribution of protease-resistant prion protein in fatal familial insomnia
    • Parchi P, Castellani R, Cortelli P, et al. Regional distribution of protease-resistant prion protein in fatal familial insomnia. Ann Neurol 1995; 38:21-29.
    • (1995) Ann Neurol , vol.38 , pp. 21-29
    • Parchi, P.1    Castellani, R.2    Cortelli, P.3
  • 103
    • 0025287947 scopus 로고
    • Codon 129 changes in the prion protein gene in Caucasians
    • Owen F, Poulter M, Collinge J, Crow TJ. Codon 129 changes in the prion protein gene in Caucasians. Am JHum Genet 1990; 46:1215-1216.
    • (1990) Am JHum Genet , vol.46 , pp. 1215-1216
    • Owen, F.1    Poulter, M.2    Collinge, J.3    Crow, T.J.4
  • 104
    • 0025820942 scopus 로고
    • Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease
    • Palmer MS, Dryden AJ, Hughes JT, Collinge J. Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature 1991; 352:340-342.
    • (1991) Nature , vol.352 , pp. 340-342
    • Palmer, M.S.1    Dryden, A.J.2    Hughes, J.T.3    Collinge, J.4
  • 105
    • 2442735162 scopus 로고    scopus 로고
    • Genetic basis of Creutzfeldt-Jakob disease in the United Kingdom: A systematic analysis of predisposing mutations and allelic variation in the PRNP gene
    • Windl O, Dempster M, Estibeiro JP, et al. Genetic basis of Creutzfeldt-Jakob disease in the United Kingdom: a systematic analysis of predisposing mutations and allelic variation in the PRNP gene. Hum Genet 1996; 98:259-264.
    • (1996) Hum Genet , vol.98 , pp. 259-264
    • Windl, O.1    Dempster, M.2    Estibeiro, J.P.3
  • 107
    • 0028000953 scopus 로고
    • Polymorphisms of the prion protein gene in Italian patients with CreutzfeldtJakob disease
    • Salvatore M, Genuardi M, Petraroli R, et al. Polymorphisms of the prion protein gene in Italian patients with CreutzfeldtJakob disease. Hum Genet 1994; 94:375-379.
    • (1994) Hum Genet , vol.94 , pp. 375-379
    • Salvatore, M.1    Genuardi, M.2    Petraroli, R.3
  • 108
    • 0027972696 scopus 로고
    • Similar genetic susceptibility in iatrogenic and sporadic Creutzfeldt-Jakob disease
    • Deslys J-P, Marcé D, Dormont D. Similar genetic susceptibility in iatrogenic and sporadic Creutzfeldt-Jakob disease. J Gen Virol 1994; 75:23-27.
    • (1994) J Gen Virol , vol.75 , pp. 23-27
    • Deslys, J.-P.1    Marcé, D.2    Dormont, D.3
  • 109
    • 8944259890 scopus 로고    scopus 로고
    • Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease
    • Parchi P, Castellani R, Capellari S, et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39:767-778.
    • (1996) Ann Neurol , vol.39 , pp. 767-778
    • Parchi, P.1    Castellani, R.2    Capellari, S.3
  • 110
    • 0030953939 scopus 로고    scopus 로고
    • Typing prion isoforms [letter]
    • Parchi P, Capellari S, Chen SG, et al. Typing prion isoforms [letter]. Nature 1997; 386:232-234.
    • (1997) Nature , vol.386 , pp. 232-234
    • Parchi, P.1    Capellari, S.2    Chen, S.G.3
  • 112
    • 0028351904 scopus 로고
    • Fatal familial insomnia and familial Creutzfeldt-Jakob disease: Different prion proteins determined by a DNA polymorphism
    • Monari L, Chen SG, Brown P, et al. Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism. Proc Natl Acad Sci USA 1994; 91:2839-2842.
    • (1994) Proc Natl Acad Sci USA , vol.91 , pp. 2839-2842
    • Monari, L.1    Chen, S.G.2    Brown, P.3
  • 114
  • 115
    • 0026507882 scopus 로고
    • Identification of heterogeneous PrP gene deletions in controls by detection of allele-specific heteroduplexes (DASH) [letter]
    • Vnencak-Jones CL, Phillips JA. Identification of heterogeneous PrP gene deletions in controls by detection of allele-specific heteroduplexes (DASH) [letter]. Am JHum Genet 1992; 50:871-872.
    • (1992) Am JHum Genet , vol.50 , pp. 871-872
    • Vnencak-Jones, C.L.1    Phillips, J.A.2
  • 116
    • 0027270270 scopus 로고
    • Deletions in the prion protein gene are not associated with CJD
    • Palmer MS, Mahal SP, Campbell TA, et al. Deletions in the prion protein gene are not associated with CJD. Hum Mol Genet 1993; 2:541-544.
    • (1993) Hum Mol Genet , vol.2 , pp. 541-544
    • Palmer, M.S.1    Mahal, S.P.2    Campbell, T.A.3
  • 117
    • 0028287867 scopus 로고
    • Detecting prion protein gene-mutations by denaturing gradient gel-electrophoresis
    • Fink JK, Peacock ML, Warren JT, et al. Detecting prion protein gene-mutations by denaturing gradient gel-electrophoresis. Hum Mutât 1994; 4:42-50.
    • (1994) Hum Mutât , vol.4 , pp. 42-50
    • Fink, J.K.1    Peacock, M.L.2    Warren, J.T.3
  • 118
    • 0029063658 scopus 로고
    • New variant prion protein in a Japanese family with Gerstmann-Straussler syndrome
    • Furukawa H, Kitamoto T, Tanaka Y, Tateishi J. New variant prion protein in a Japanese family with Gerstmann-Straussler syndrome. Mol Brain Res 1995; 30:385-388.
    • (1995) Mol Brain Res , vol.30 , pp. 385-388
    • Furukawa, H.1    Kitamoto, T.2    Tanaka, Y.3    Tateishi, J.4
  • 119
    • 0026061028 scopus 로고
    • New mutation in scrapie amyloid precursor gene (at codon 178) in Finnish Creutzfeldt-Jakob kindred [letter]
    • Goldfarb LG, Haltia M, Brown P, et al. New mutation in scrapie amyloid precursor gene (at codon 178) in Finnish Creutzfeldt-Jakob kindred [letter]. Lancet 1991; 337:425.
    • (1991) Lancet , vol.337 , pp. 425
    • Goldfarb, L.G.1    Haltia, M.2    Brown, P.3
  • 120
    • 0026581952 scopus 로고
    • Phenotypic characteristics of familial Creutzfeldt-Jakob disease associated with the codon 178Asn PRNP mutation
    • Brown P, Goldfarb LG, Kovanen J, et al. Phenotypic characteristics of familial Creutzfeldt-Jakob disease associated with the codon 178Asn PRNP mutation. Ann Neurol 1992; 31:282-285.
    • (1992) Ann Neurol , vol.31 , pp. 282-285
    • Brown, P.1    Goldfarb, L.G.2    Kovanen, J.3
  • 121
    • 0026552106 scopus 로고
    • Creutzfeldt-Jakob disease segregates with the codon 178Asn PRNP mutation in families of European origin
    • Goldfarb LG, Brown P, Haltia M, et al. Creutzfeldt-Jakob disease (segregates with the codon 178Asn PRNP mutation in families of European origin. Ann Neurol 1992; 31:274-281.
    • (1992) Ann Neurol , vol.31 , pp. 274-281
    • Goldfarb, L.G.1    Brown, P.2    Haltia, M.3
  • 122
    • 0026082629 scopus 로고
    • Codon 178 mutation in ethnically diverse Creutzfeldt-Jakob disease families [letter]
    • Nieto A, Goldfarb LG, Brown P, et al. Codon 178 mutation in ethnically diverse Creutzfeldt-Jakob disease families [letter]. Lancet 1991; 337:622-623.
    • (1991) Lancet , vol.337 , pp. 622-623
    • Nieto, A.1    Goldfarb, L.G.2    Brown, P.3
  • 123
  • 124
    • 0019801464 scopus 로고
    • The familial occurrence of Creutzfeldt-Jakob disease and Alzheimer's disease
    • Masters CL, Gajdusek DC, Gibbs CJ, The familial occurrence of Creutzfeldt-Jakob disease and Alzheimer's disease. Brain 1981; 104:535-558.
    • (1981) Brain , vol.104 , pp. 535-558
    • Masters, C.L.1    Gajdusek, D.C.2    Gibbs, C.J.3    Jr4
  • 126
    • 0023879939 scopus 로고
    • Descriptive epidemiology of CreutzfeldtJakob disease in Finland
    • Kovanen J, Haltia M. Descriptive epidemiology of CreutzfeldtJakob disease in Finland. Acta Neurol Scand 1988; 77:474-480.
    • (1988) Acta Neurol Scand , vol.77 , pp. 474-480
    • Kovanen, J.1    Haltia, M.2
  • 128
    • 0027185917 scopus 로고
    • Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Sträussler syndrome
    • Kitamoto T, Ohta M, Doh-ura K, et al. Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Sträussler syndrome. Biochem Biophys Res Commun 1993; 191:709-714.
    • (1993) Biochem Biophys Res Commun , vol.191 , pp. 709-714
    • Kitamoto, T.1    Ohta, M.2    Doh-ura, K.3
  • 129
    • 15644366144 scopus 로고    scopus 로고
    • Familial spongiform encephalopathy associated with a novel prion protein gene mutation
    • Nitrini R, Rosemberg S, Passos-Bueno MR, et al. Familial spongiform encephalopathy associated with a novel prion protein gene mutation. Ann Neurol 1997; 42:138-146.
    • (1997) Ann Neurol , vol.42 , pp. 138-146
    • Nitrini, R.1    Rosemberg, S.2    Passos-Bueno, M.R.3
  • 130
    • 0024995430 scopus 로고
    • Mutation in codon 200 of scrapie amyloid protein gene in two clusters of CreutzfeldtJakob disease in Slovakia [letter]
    • Goldfarb LG, Mitrova E, Brown P, et al. Mutation in codon 200 of scrapie amyloid protein gene in two clusters of CreutzfeldtJakob disease in Slovakia [letter]. Lancet 1990; 336:514-515.
    • (1990) Lancet , vol.336 , pp. 514-515
    • Goldfarb, L.G.1    Mitrova, E.2    Brown, P.3
  • 131
    • 0025869213 scopus 로고
    • Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease
    • Hsiao K, Meiner Z, Kahana E, et al. Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. N Engl JMed 1991; 324:1091-1097.
    • (1991) N Engl JMed , vol.324 , pp. 1091-1097
    • Hsiao, K.1    Meiner, Z.2    Kahana, E.3
  • 132
    • 0028782024 scopus 로고
    • Mutation in codon 200 and polymorphism in codon 129 of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease
    • Gabizon R, Rosenman H, Meiner Z, et al. Mutation in codon 200 and polymorphism in codon 129 of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease. Phil Trans R Sac Land B Biol Sci 1994; 343:385-390.
    • (1994) Phil Trans R Sac Land B Biol Sci , vol.343 , pp. 385-390
    • Gabizon, R.1    Rosenman, H.2    Meiner, Z.3
  • 133
    • 0025918142 scopus 로고
    • Creutzfeldt-Jacob disease associated with the PRNP codon 20011 mutation: An analysis of 45 families
    • Goldfarb LG, Brown P, Mitrova E, et al. Creutzfeldt-Jacob disease associated with the PRNP codon 20011 mutation: an analysis of 45 families. Eur J Epidemiol 1991; 7:477-486.
    • (1991) Eur J Epidemiol , vol.7 , pp. 477-486
    • Goldfarb, L.G.1    Brown, P.2    Mitrova, E.3
  • 134
    • 0026662717 scopus 로고
    • Familial CreutzfeldtJakob disease (codon 200 mutation) with supranuclear palsy
    • Bertoni JM, Brown P, Goldfarb LG, et al. Familial CreutzfeldtJakob disease (codon 200 mutation) with supranuclear palsy. JAMA 1992; 268:2413-2415.
    • (1992) JAMA , vol.268 , pp. 2413-2415
    • Bertoni, J.M.1    Brown, P.2    Goldfarb, L.G.3
  • 135
    • 0028206519 scopus 로고
    • Japanese family with Creutzfeldt-Jakob disease with codon 200 point mutation of the prion protein gene
    • Inoue I, Kitamoto T, Doh-ura K, et al. Japanese family with Creutzfeldt-Jakob disease with codon 200 point mutation of the prion protein gene. Neurology 1994; 44:299-301.
    • (1994) Neurology , vol.44 , pp. 299-301
    • Inoue, I.1    Kitamoto, T.2    Doh-ura, K.3
  • 136
    • 0024992359 scopus 로고
    • Mutation in codon 200 of scrapie amyloid precursor gene linked to Creutzfeldt-Jakob disease in Sephardic Jews of Libyan and non-Libyan origin [letter]
    • Goldfarb L, Korczyn A, Brown P, et al. Mutation in codon 200 of scrapie amyloid precursor gene linked to Creutzfeldt-Jakob disease in Sephardic Jews of Libyan and non-Libyan origin [letter]. Lancet 1990; 336:637-638.
    • (1990) Lancet , vol.336 , pp. 637-638
    • Goldfarb, L.1    Korczyn, A.2    Brown, P.3
  • 137
    • 0026481859 scopus 로고
    • Familial CreutzfeldtJakob disease in Chile is associated with the codon 200 mutation of the PRNP amyloid precursor gene on chromosome 20
    • Brown P, Gâlvez S, Goldfarb LG, et al. Familial CreutzfeldtJakob disease in Chile is associated with the codon 200 mutation of the PRNP amyloid precursor gene on chromosome 20. J Neurol Sci 1992; 112:65-67.
    • (1992) J Neurol Sci , vol.112 , pp. 65-67
    • Brown, P.1    Gâlvez, S.2    Goldfarb, L.G.3
  • 141
    • 0027443351 scopus 로고
    • Clinical heterogeneity and unusual presentations of Creutzfeldt-Jakob disease in Jewish patients with the PRNP codon 200 mutation
    • Chapman J, Brown P, Goldfarb LG, et al. Clinical heterogeneity and unusual presentations of Creutzfeldt-Jakob disease in Jewish patients with the PRNP codon 200 mutation. J Neurol Neurosurg Psychiatry 1993; 56:1109-1112.
    • (1993) J Neurol Neurosurg Psychiatry , vol.56 , pp. 1109-1112
    • Chapman, J.1    Brown, P.2    Goldfarb, L.G.3
  • 142
    • 0026703146 scopus 로고
    • Demyelinating peripheral neuropathy in Creutzfeldt-Jakob disease
    • Neufeld MY, Josiphov J, Korczyn AD. Demyelinating peripheral neuropathy in Creutzfeldt-Jakob disease. Muscle Nerve 1992; 15:1234-1239.
    • (1992) Muscle Nerve , vol.15 , pp. 1234-1239
    • Neufeld, M.Y.1    Josiphov, J.2    Korczyn, A.D.3
  • 143
    • 0029877723 scopus 로고    scopus 로고
    • Fatal insomnia in a case of familial Creutzfeldt-Jakob disease with the codon 200' mutation
    • Chapman J, Arlazoroff A, Goldfarb LG, et al. Fatal insomnia in a case of familial Creutzfeldt-Jakob disease with the codon 200' mutation. Neurology 1996; 46:758-761.
    • (1996) Neurology , vol.46 , pp. 758-761
    • Chapman, J.1    Arlazoroff, A.2    Goldfarb, L.G.3
  • 144
    • 0028145428 scopus 로고
    • The risk of developing Creutzfeldt-Jakob disease in subjects with the PRNP gene codon 200 point mutation
    • Chapman J, Ben-Israel J, Goldhammer Y, Korczyn AD. The risk of developing Creutzfeldt-Jakob disease in subjects with the PRNP gene codon 200 point mutation. Neurology 1994; 44:1683-1686.
    • (1994) Neurology , vol.44 , pp. 1683-1686
    • Chapman, J.1    Ben-Israel, J.2    Goldhammer, Y.3    Korczyn, A.D.4
  • 145
    • 0026687389 scopus 로고
    • Transmission of spongiform encephalopathies from a familial Creutzfeldt-Jakob disease patient of Jewish Libyan origin carrying the PRNP codon 200 mutation
    • Chapman J, Brown P, Rabey JM, et al. Transmission of spongiform encephalopathies from a familial Creutzfeldt-Jakob disease patient of Jewish Libyan origin carrying the PRNP codon 200 mutation. Neurology 1992; 42:1249-1250.
    • (1992) Neurology , vol.42 , pp. 1249-1250
    • Chapman, J.1    Brown, P.2    Rabey, J.M.3
  • 146
    • 0007566581 scopus 로고
    • Identification of a new mutation of the prion protein gene at codon 208 in a patient with Creutzfeldt-Jakob disease [abstract]
    • Mastrianni JA, lannicola C, Myers R, et al. Identification of a new mutation of the prion protein gene at codon 208 in a patient with Creutzfeldt-Jakob disease [abstract]. Neurology 1995; 45[Suppl]:201.
    • (1995) Neurology , vol.45 , pp. 201
    • Mastrianni, J.A.1    Lannicola, C.2    Myers, R.3
  • 147
    • 0027378249 scopus 로고
    • A new point mutation of the prion protein gene in familial and sporadic cases of Creutzfeldt-Jakob disease
    • Pocchiari M, Salvatore M, Cutruzzola F, et al. A new point mutation of the prion protein gene in familial and sporadic cases of Creutzfeldt-Jakob disease. Ann Neurol 1993; 34: 802-807.
    • (1993) Ann Neurol , vol.34 , pp. 802-807
    • Pocchiari, M.1    Salvatore, M.2    Cutruzzola, F.3
  • 148
    • 0027425690 scopus 로고
    • A new point mutation in thé prion protein gene at codon 210 in CreutzfeldtJakob disease
    • Ripoll L, Laplanche J-L, Salzmann M, et al. A new point mutation in thé prion protein gene at codon 210 in CreutzfeldtJakob disease. Neurology 1993; 43:1934-1938.
    • (1993) Neurology , vol.43 , pp. 1934-1938
    • Ripoll, L.1    Laplanche, J.-L.2    Salzmann, M.3
  • 150
    • 0024530897 scopus 로고
    • Insertion in prion protein gene in familial Creutzfeldt-Jakob disease [letter]
    • Owen F, Poulter M, Lofthouse R, et al. Insertion in prion protein gene in familial Creutzfeldt-Jakob disease [letter]. Lancet 1989; 1:51-52.
    • (1989) Lancet , vol.1 , pp. 51-52
    • Owen, F.1    Poulter, M.2    Lofthouse, R.3
  • 151
    • 0025055393 scopus 로고
    • An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease
    • Owen F, Poulter M, Shah T, et al. An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease. Mol Brain Res 1990; 7:273-276.
    • (1990) Mol Brain Res , vol.7 , pp. 273-276
    • Owen, F.1    Poulter, M.2    Shah, T.3
  • 154
    • 0025330687 scopus 로고
    • Prion dementia without characteristic pathology
    • Collinge J, Owen F, Poulter H, et al. Prion dementia without characteristic pathology. Lancet 1990; 336:7-9.
    • (1990) Lancet , vol.336 , pp. 7-9
    • Collinge, J.1    Owen, F.2    Poulter, H.3
  • 156
    • 0028946529 scopus 로고
    • Inherited CreutzfeldtJakob disease in a British family associated with a novel 144 base pair insertion of the prion protein gene
    • Nicholl D, Windl O, de Suva R, et al. Inherited CreutzfeldtJakob disease in a British family associated with a novel 144 base pair insertion of the prion protein gene. J Neurol Neurosurg Psychiatr 1995; 58:65-69.
    • (1995) J Neurol Neurosurg Psychiatr , vol.58 , pp. 65-69
    • Nicholl, D.1    Windl, O.2    De Suva, R.3
  • 157
    • 0029004216 scopus 로고
    • Prion disease with 144 base pair insertion in a Japanese family Une
    • Oda T, Kitamoto T, Tateishi J, et al. Prion disease with 144 base pair insertion in a Japanese family Une. Acta Neuropathol 1995; 90:80-86.
    • (1995) Acta Neuropathol , vol.90 , pp. 80-86
    • Oda, T.1    Kitamoto, T.2    Tateishi, J.3
  • 158
    • 0029794281 scopus 로고    scopus 로고
    • Familial Creutzfeldt-Jakob disease with a five-repeat octapeptide insert mutation
    • Cochran EJ, Bennett DA, Cervenâkovâ L, et al. Familial Creutzfeldt-Jakob disease with a five-repeat octapeptide insert mutation. Neurology 1996; 47:727-733
    • (1996) Neurology , vol.47 , pp. 727-733
    • Cochran, E.J.1    Bennett, D.A.2    Cervenâkovâ, L.3
  • 159
    • 0030756021 scopus 로고    scopus 로고
    • Familial prion disease with a novel 144-bp insertion in thé prion protein gene in a Basque family
    • Capellari S, Vital C, Parchi P, et al. Familial prion disease with a novel 144-bp insertion in thé prion protein gene in a Basque family. Neurology 1997; 49:133-141.
    • (1997) Neurology , vol.49 , pp. 133-141
    • Capellari, S.1    Vital, C.2    Parchi, P.3
  • 160
    • 0027480960 scopus 로고
    • A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
    • The Huntington's Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 1993; 72:971-983.
    • (1993) Cell , vol.72 , pp. 971-983
    • Group, T.H.1
  • 163
    • 0025885702 scopus 로고
    • Transmissible familial Creutzfeldt-Jakob disease associated with five, seven, and eight extra octapeptide coding repeats in the PRNP gene
    • Goldfarb LG, Brown P, McCombie WR, et al. Transmissible familial Creutzfeldt-Jakob disease associated with five, seven, and eight extra octapeptide coding repeats in the PRNP gene. ProcNatlAcad Sci USA 1991; 88:10926-10930.
    • (1991) ProcNatlAcad Sci USA , vol.88 , pp. 10926-10930
    • Goldfarb, L.G.1    Brown, P.2    McCombie, W.R.3
  • 164
    • 0029598460 scopus 로고
    • Hypokinesia and presenile dementia in a Dutch family with a novel insertion in the prion protein gene
    • van Gool WA, Hensels GW, Hoogerwaard EM, et al. Hypokinesia and presenile dementia in a Dutch family with a novel insertion in the prion protein gene. Brain 1995; 118:1565-1571.
    • (1995) Brain , vol.118 , pp. 1565-1571
    • Gool, W.A.1    Hensels, G.W.2    Hoogerwaard, E.M.3
  • 165
    • 0031456947 scopus 로고    scopus 로고
    • Structure of the recombinant full-length hamster prion protein PrP(29-231): The N terminus is highly flexible
    • Donne DG, Viles JH, Groth D, et al. Structure of the recombinant full-length hamster prion protein PrP(29-231): the N terminus is highly flexible. Proc Natl Acad Sci USA 1997; 94:13452-13457.
    • (1997) Proc Natl Acad Sci USA , vol.94 , pp. 13452-13457
    • Donne, D.G.1    Viles, J.H.2    Groth, D.3
  • 166
    • 0026609576 scopus 로고
    • Prion protein mutation at codon 102 in an Italian family with GerstmannSträussler-Scheinker syndrome
    • Kretzschmar HA, Kufer P, Riethmüller G, et al. Prion protein mutation at codon 102 in an Italian family with GerstmannSträussler-Scheinker syndrome. Neurology 1992; 42:809-810.
    • (1992) Neurology , vol.42 , pp. 809-810
    • Kretzschmar, H.A.1    Kufer, P.2    Riethmüller, G.3
  • 167
    • 0027730422 scopus 로고
    • An Israeli family with Gerstmann-Sträussler-Scheinker disease manifesting the codon 102 mutation in the prion protein gene
    • Goldhammer Y, Gabizon R, Meiner Z, Sadeh M. An Israeli family with Gerstmann-Sträussler-Scheinker disease manifesting the codon 102 mutation in the prion protein gene. Neurology 1993; 43:2718-2719.
    • (1993) Neurology , vol.43 , pp. 2718-2719
    • Goldhammer, Y.1    Gabizon, R.2    Meiner, Z.3    Sadeh, M.4
  • 168
    • 0024473899 scopus 로고
    • Pro - Leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Sträussler syndrome
    • Doh-ura K, Tateishi J, Sasaki H, et al. Pro - Leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Sträussler syndrome. Biochem Biophys Res Commun 1989; 163:974-979.
    • (1989) Biochem Biophys Res Commun , vol.163 , pp. 974-979
    • Doh-ura, K.1    Tateishi, J.2    Sasaki, H.3
  • 169
    • 0024467653 scopus 로고
    • Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-SträusslerScheinker's syndrome
    • Goldgaber D, Goldfarb LG, Brown P, et al. Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-SträusslerScheinker's syndrome. Exp Neurol 1989; 106:204-206.
    • (1989) Exp Neurol , vol.106 , pp. 204-206
    • Goldgaber, D.1    Goldfarb, L.G.2    Brown, P.3
  • 170
    • 0028989819 scopus 로고
    • Gerstmann-SträusslerScheinker disease with mutation at codon 102 and methionine at codon 129 of PRNP in previously unreported patients
    • Young K, Jones CK, Piccardo P, et al. Gerstmann-SträusslerScheinker disease with mutation at codon 102 and methionine at codon 129 of PRNP in previously unreported patients. Neurology 1995; 45:1127-1134.
    • (1995) Neurology , vol.45 , pp. 1127-1134
    • Young, K.1    Jones, C.K.2    Piccardo, P.3
  • 171
    • 0025906297 scopus 로고
    • Prion protein mutation in family first reported by Gerstmann, Sträussler, and Scheinker [letter]
    • Kretzschmar HA, Honold G, Seitelberger F, et al. Prion protein mutation in family first reported by Gerstmann, Sträussler, and Scheinker [letter]. Lancet 1991; 337:1160.
    • (1991) Lancet , vol.337 , pp. 1160
    • Kretzschmar, H.A.1    Honold, G.2    Seitelberger, F.3
  • 172
    • 0026065187 scopus 로고
    • Support of linkage of Gerstmann-Sträussler-Scheinker syndrome to the prion protein gene on chromosome 20pl2-pter
    • Speer MC, Goldgaber D, Goldfarb LG, et al. Support of linkage of Gerstmann-Sträussler-Scheinker syndrome to the prion protein gene on chromosome 20pl2-pter. Genomics 1991; 9:366-368.
    • (1991) Genomics , vol.9 , pp. 366-368
    • Speer, M.C.1    Goldgaber, D.2    Goldfarb, L.G.3
  • 173
    • 0028990981 scopus 로고
    • The original Gerstmann-Sträussler-Scheinker family of Austria: Divergent clinicopathological phenotypes but constant PrP genotype
    • Hainfelmer JA, Brantner-Inthaler S, Cervenakova L, et al. The original Gerstmann-Sträussler-Scheinker family of Austria: divergent clinicopathological phenotypes but constant PrP genotype. Brain Pathol 1995; 5:201-211.
    • (1995) Brain Pathol , vol.5 , pp. 201-211
    • Hainfelmer, J.A.1    Brantner-Inthaler, S.2    Cervenakova, L.3
  • 174
    • 0028989031 scopus 로고
    • The dementia of Gerstmann-Sträussler-Scheinker syndrome: Clinical variability demonstrated by two case reports
    • Lyketsos CG, Kraus M. The dementia of Gerstmann-Sträussler-Scheinker syndrome: clinical variability demonstrated by two case reports. J Neuropsychiatr Clin Neurosci 1995; 7:239-242.
    • (1995) J Neuropsychiatr Clin Neurosci , vol.7 , pp. 239-242
    • Lyketsos, C.G.1    Kraus, M.2
  • 175
    • 0030925860 scopus 로고    scopus 로고
    • A Japanese family with a variant of Gerstmann-Sträussler-Scheinker disease
    • Tanaka Y, Minematsu K, Moriyasu H, et al. A Japanese family with a variant of Gerstmann-Sträussler-Scheinker disease. J Neurol Neurosurg Psychiatr 1997; 62:454-457.
    • (1997) J Neurol Neurosurg Psychiatr , vol.62 , pp. 454-457
    • Tanaka, Y.1    Minematsu, K.2    Moriyasu, H.3
  • 176
    • 0025681138 scopus 로고
    • Spontaneous neurodegencration in transgenic mice with mutant prion protein
    • Hsiao KK, Scott M, Foster D, et al. Spontaneous neurodegencration in transgenic mice with mutant prion protein. Science 1990; 250:1587-1590.
    • (1990) Science , vol.250 , pp. 1587-1590
    • Hsiao, K.K.1    Scott, M.2    Foster, D.3
  • 177
    • 0028608963 scopus 로고
    • Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein
    • Hsiao KK, Groth D, Scott M, et al. Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. ProcNatlAcad Sci USA 1994;91: 9126-9130.
    • (1994) ProcNatlAcad Sci USA , vol.91 , pp. 9126-9130
    • Hsiao, K.K.1    Groth, D.2    Scott, M.3
  • 178
    • 0019778656 scopus 로고
    • Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies
    • Masters CL, Gajdusek DC, Gibbs CJ, Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies. Brain 1981; 104:559-588.
    • (1981) Brain , vol.104 , pp. 559-588
    • Masters, C.L.1    Gajdusek, D.C.2    Gibbs, C.J.3    Jr4
  • 179
    • 0026453980 scopus 로고
    • Gerstmann-SträusslerScheinker syndrome-a variant type: Amyloid plaques and Alzheimer's neurofibrillary tangles in cerebral cortex
    • Amano N, Yagishita S, Yokoi S, et al. Gerstmann-SträusslerScheinker syndrome-a variant type: amyloid plaques and Alzheimer's neurofibrillary tangles in cerebral cortex. Acta Neuropathol 1992; 84:15-23.
    • (1992) Acta Neuropathol , vol.84 , pp. 15-23
    • Amano, N.1    Yagishita, S.2    Yokoi, S.3
  • 180
    • 0026331619 scopus 로고
    • An autopsy case of Gerstmann-Sträussler-Scheinker's disease with spastic paraplegia as its principal feature
    • Nakazato Y, Ohno R, Negishi T, et al. An autopsy case of Gerstmann-Sträussler-Scheinker's disease with spastic paraplegia as its principal feature. Clin Neurol 1991; 31:987-992.
    • (1991) Clin Neurol , vol.31 , pp. 987-992
    • Nakazato, Y.1    Ohno, R.2    Negishi, T.3
  • 181
    • 0027729337 scopus 로고
    • A missense mutation at codon 105 with codon 129 polymorphism of the prion protein gene in a new variant of Gerstmann-Straussler-Scheinker disease
    • Yamada M, Itoh Y, Fujigasaki H, et al. A missense mutation at codon 105 with codon 129 polymorphism of the prion protein gene in a new variant of Gerstmann-Straussler-Scheinker disease. Neurology 1993; 43:2723-2724.
    • (1993) Neurology , vol.43 , pp. 2723-2724
    • Yamada, M.1    Itoh, Y.2    Fujigasaki, H.3
  • 182
    • 0027497304 scopus 로고
    • A new inherited prion disease (PrP-P105L mutation) showing spastic paraparesis
    • Kitamoto T, Amano N, Terao Y, et al. A new inherited prion disease (PrP-P105L mutation) showing spastic paraparesis. Ann Neurol 1993; 34:808-813.
    • (1993) Ann Neurol , vol.34 , pp. 808-813
    • Kitamoto, T.1    Amano, N.2    Terao, Y.3
  • 183
    • 0025847141 scopus 로고
    • A prion protein variant in a family with the telencephalic form of GerstmannStraussler-Scheinker syndrome
    • Hsiao KK, Cass C, Schellenberg GD, et al. A prion protein variant in a family with the telencephalic form of GerstmannStraussler-Scheinker syndrome. Neurology 1991; 41:681-684.
    • (1991) Neurology , vol.41 , pp. 681-684
    • Hsiao, K.K.1    Cass, C.2    Schellenberg, G.D.3
  • 184
    • 0029398569 scopus 로고
    • Prion disease (PrP-A117V) presenting with ataxia instead of dementia
    • Mastrianni JA, Curtis MT, Oberholtzer JC, et al. Prion disease (PrP-A117V) presenting with ataxia instead of dementia. Neurology 1995; 45:2042-2050.
    • (1995) Neurology , vol.45 , pp. 2042-2050
    • Mastrianni, J.A.1    Curtis, M.T.2    Oberholtzer, J.C.3
  • 186
    • 0024324177 scopus 로고
    • Familial dementia with PrP-positive amyloid plaques: A variant of Gerstmann-Sträussler syndrome
    • Nochlin D, Sumi SM, Bird TD, et al. Familial dementia with PrP-positive amyloid plaques: a variant of Gerstmann-Sträussler syndrome. Neurology 1989; 39:910-918.
    • (1989) Neurology , vol.39 , pp. 910-918
    • Nochlin, D.1    Sumi, S.M.2    Bird, T.D.3
  • 187
    • 0025119522 scopus 로고
    • Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-Straussler-Scheinker syndrome
    • Tateishi J, Kitamoto T, Doh-ura K, et al. Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-Straussler-Scheinker syndrome. Neurology 1990; 40: 1578-1581.
    • (1990) Neurology , vol.40 , pp. 1578-1581
    • Tateishi, J.1    Kitamoto, T.2    Doh-ura, K.3
  • 188
    • 0004882124 scopus 로고
    • Alsatian variant of codon 117 form of Gerstmann-Straussler-Scheinker syndrome: Autopsie study of 3 cases
    • Mohr M, Tranchant C, Heldt N, et al. Alsatian variant of codon 117 form of Gerstmann-Straussler-Scheinker syndrome: autopsie study of 3 cases. Brain Pathol 1994; 4:524.
    • (1994) Brain Pathol , vol.4 , pp. 524
    • Mohr, M.1    Tranchant, C.2    Heldt, N.3
  • 189
    • 0032488777 scopus 로고    scopus 로고
    • A transmembrane form of the prion protein in neurodegenerative disease
    • Hegde RS, Mastrianni JA, Scott MR, et al. A transmembrane form of the prion protein in neurodegenerative disease. Science 1998; 279:827-834.
    • (1998) Science , vol.279 , pp. 827-834
    • Hegde, R.S.1    Mastrianni, J.A.2    Scott, M.R.3
  • 190
    • 0027236933 scopus 로고
    • An amber mutation of prion protein in Gerstmann-Sträussler syndrome with mutant PrP plaques
    • Kitamoto T, lizuka R, Tateishi J. An amber mutation of prion protein in Gerstmann-Sträussler syndrome with mutant PrP plaques. Biochem Biophys Res Commun 1993; 192:525-531.
    • (1993) Biochem Biophys Res Commun , vol.192 , pp. 525-531
    • Kitamoto, T.1    Lizuka, R.2    Tateishi, J.3
  • 191
    • 0026849545 scopus 로고
    • Linkage of the Indiana kindred of Gerstmann-Straussler-Scheinker disease to the prion protein gene
    • Dlouhy SR, Hsiao K, Farlow MR, et al. Linkage of the Indiana kindred of Gerstmann-Straussler-Scheinker disease to the prion protein gene. Nat Genet 1992; 1:64-67.
    • (1992) Nat Genet , vol.1 , pp. 64-67
    • Dlouhy, S.R.1    Hsiao, K.2    Farlow, M.R.3
  • 195
    • 0028984802 scopus 로고
    • Gerstmann-Straussler-Scheinker disease and the Indiana kindred
    • Ghetti B, Dlouhy SR, Giaccone G, et al. Gerstmann-Straussler-Scheinker disease and the Indiana kindred. Brain Pathol 1995; 5:61-75.
    • (1995) Brain Pathol , vol.5 , pp. 61-75
    • Ghetti, B.1    Dlouhy, S.R.2    Giaccone, G.3
  • 196
    • 0026849947 scopus 로고
    • Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles
    • Hsiao K, Dlouhy S, Farlow MR, et al. Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles. Nat Genet 1992; 1:68-71.
    • (1992) Nat Genet , vol.1 , pp. 68-71
    • Hsiao, K.1    Dlouhy, S.2    Farlow, M.R.3
  • 197
    • 0028004290 scopus 로고
    • 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, et al. Amyloid fibrils in Gerstmann-Sträussler-Scheinker disease (Indiana and Swedish kindreds) express only PrP peptides encoded by the mutant allele. Cell 1994; 79:695-703.
    • (1994) Cell , vol.79 , pp. 695-703
    • Tagliavini, F.1    Prelli, F.2    Porro, M.3
  • 198
    • 0022633025 scopus 로고
    • Precautions in handling tissues, fluids, and other contaminated materials from patients with documented or suspected Creutzfeldt-Jakob disease
    • Rosenberg RN, White LL, III, Brown P, et al. Precautions in handling tissues, fluids, and other contaminated materials from patients with documented or suspected Creutzfeldt-Jakob disease. Ann Neurol 1986; 19:75-77.
    • (1986) Ann Neurol , vol.19 , pp. 75-77
    • Rosenberg, R.N.1    White, L.L.2    Brown, P.3
  • 199
    • 0029840653 scopus 로고    scopus 로고
    • The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies
    • Hsich G, Kenney K, Gibbs CJ, et al. The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. NEngl J Med 1996; 335:924-930.
    • (1996) NEngl J Med , vol.335 , pp. 924-930
    • Hsich, G.1    Kenney, K.2    Gibbs, C.J.3
  • 200
    • 0031914675 scopus 로고    scopus 로고
    • Detection of 14-3-3 protein in the cerebrospinal fluid supports the diagnosis of Creutzfeldt-Jakob disease
    • Zerr I, Bodemer M, Gefeller O, et al. Detection of 14-3-3 protein in the cerebrospinal fluid supports the diagnosis of Creutzfeldt-Jakob disease. Ann Neurol 1998; 43:32-40.
    • (1998) Ann Neurol , vol.43 , pp. 32-40
    • Zerr, I.1    Bodemer, M.2    Gefeller, O.3
  • 201
    • 0025971881 scopus 로고
    • Chemoprophylaxis of scrapie in mice
    • Diringer H, Ehlers B. Chemoprophylaxis of scrapie in mice. JGen Viral 1991; 72:457-460.
    • (1991) JGen Viral , vol.72 , pp. 457-460
    • Diringer, H.1    Ehlers, B.2
  • 202
    • 0026050318 scopus 로고
    • Experimental drug treatment of scrapie: A pathogenetic basis for rationale therapeutics
    • Pocchiari M, Salvatore M, Ladogana A, et al. Experimental drug treatment of scrapie: a pathogenetic basis for rationale therapeutics. Eur J Epidemiol 1991; 7:556-561.
    • (1991) Eur J Epidemiol , vol.7 , pp. 556-561
    • Pocchiari, M.1    Salvatore, M.2    Ladogana, A.3
  • 203
    • 0000672037 scopus 로고
    • A prion protein amino acid substitution in ataxic Gerstmann-Sträussler syndrome
    • Hsiao KK, Doh-ura K, Kitamoto T, et al. A prion protein amino acid substitution in ataxic Gerstmann-Sträussler syndrome. Ann Neurol 1989; 26:137.
    • (1989) Ann Neurol , vol.26 , pp. 137
    • Hsiao, K.K.1    Doh-ura, K.2    Kitamoto, T.3
  • 204
    • 0029026751 scopus 로고
    • Two novel insertions in the prion protein gene in patients with late-onset dementia
    • Laplanche JL, Delasnerie-Lauprêtre N, Brandel JP, et al. Two novel insertions in the prion protein gene in patients with late-onset dementia. Hum Mol Genet 1995; 4:1109-1111.204. Goldfarb LG, Brown P, Little BW, et al. A new (two-repeat) octapeptide coding insert mutation in Creutzfeldt-Jakob disease. Neurology 1993; 43:2392-2394.
    • (1995) Hum Mol Genet , vol.4 , pp. 1109-1111
    • Laplanche, J.L.1    Delasnerie-Lauprêtre, N.2    Brandel, J.P.3
  • 205
    • 0029874720 scopus 로고    scopus 로고
    • A prion disease with a novel 96-base pair insertional mutation in the prion protein gene
    • Campbell TA, Palmer MS, Will RG, et al. A prion disease with a novel 96-base pair insertional mutation in the prion protein gene. Neurology 1996; 46:761-766.
    • (1996) Neurology , vol.46 , pp. 761-766
    • Campbell, T.A.1    Palmer, M.S.2    Will, R.G.3
  • 206
    • 0026606082 scopus 로고
    • Atypical Creutzfeldt-Jakob disease in an American family with an insert mutation in the PRNP amyloid precursor gene
    • Brown P, Goldfarb LG, McCombie WR, et al. Atypical Creutzfeldt-Jakob disease in an American family with an insert mutation in the PRNP amyloid precursor gene. Neurology 1992; 42:422-427.
    • (1992) Neurology , vol.42 , pp. 422-427
    • Brown, P.1    Goldfarb, L.G.2    McCombie, W.R.3
  • 207
    • 0342495573 scopus 로고
    • A case of presenile dementia with a 168 base pair insertion in prion protein gene
    • Mizushima S, Ishii K, Nishimaru T. A case of presenile dementia with a 168 base pair insertion in prion protein gene. Dementia 1994; 148:380-390.
    • (1994) Dementia , vol.148 , pp. 380-390
    • Mizushima, S.1    Ishii, K.2    Nishimaru, T.3
  • 208
    • 0026644052 scopus 로고
    • An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann-Straussler-Scheinker family
    • Goldfarb LG, Brown P, Vrbovskâ A, et al. An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann-Straussler-Scheinker family. J Neurol Sci 1992; 111:189-194.
    • (1992) J Neurol Sci , vol.111 , pp. 189-194
    • Goldfarb, L.G.1    Brown, P.2    Vrbovskâ, A.3
  • 209
    • 0026513044 scopus 로고
    • A dementing illness associated with a novel insertion in the prion protein gene
    • Owen F, Poulter M, Collinge J, et al. A dementing illness associated with a novel insertion in the prion protein gene. Mol Brain Res 1992; 13:155-157.
    • (1992) Mol Brain Res , vol.13 , pp. 155-157
    • Owen, F.1    Poulter, M.2    Collinge, J.3
  • 210
    • 0028820863 scopus 로고
    • Prion disease associated with a novel nine octapeptide repeat insertion in the PRNP gene
    • Krasemann S, Zerr I, Weber T, et al. Prion disease associated with a novel nine octapeptide repeat insertion in the PRNP gene. Mol Brain Res 1995; 34:173-176.
    • (1995) Mol Brain Res , vol.34 , pp. 173-176
    • Krasemann, S.1    Zerr, I.2    Weber, T.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.