-
1
-
-
0001168222
-
Rida, a chronic encephalitis of sheep with general remarks on infections which develop slowly and some of their special characteristics
-
Sigurdsson B (1954) Rida, a chronic encephalitis of sheep with general remarks on infections which develop slowly and some of their special characteristics. Br Vet J 110:341-354
-
(1954)
Br Vet J
, vol.110
, pp. 341-354
-
-
Sigurdsson, B.1
-
2
-
-
0017643758
-
Unconventional viruses and the origin and disappearance of kuru
-
Gajdusek DC (1977) Unconventional viruses and the origin and disappearance of kuru. Science 197:943-960
-
(1977)
Science
, vol.197
, pp. 943-960
-
-
Gajdusek, D.C.1
-
3
-
-
0009114182
-
Subacute spongiform virus encephalopathies caused by unconventional viruses
-
Maramorosch K, McKelvey JJ, Jr, eds, Academic Press, Orlando
-
Gajdusek DC (1985) Subacute spongiform virus encephalopathies caused by unconventional viruses, in Subviral Pathogens of Plants and Animals: Viroids and Prions (Maramorosch K, McKelvey JJ, Jr, eds), pp 483-544. Academic Press, Orlando
-
(1985)
Subviral Pathogens of Plants and Animals: Viroids and Prions
, pp. 483-544
-
-
Gajdusek, D.C.1
-
4
-
-
0014211846
-
Does the agent of scrapie replicate without nucleic acid?
-
Alper T, Cramp WA, Haig DA, Clarke MC (1967) Does the agent of scrapie replicate without nucleic acid? Nature 214:764-766
-
(1967)
Nature
, vol.214
, pp. 764-766
-
-
Alper, T.1
Cramp, W.A.2
Haig, D.A.3
Clarke, M.C.4
-
5
-
-
0015318003
-
Scrapie: A prototype slow infection
-
Hunter GD (1972) Scrapie: a prototype slow infection. J Infect Dis 125:427-440
-
(1972)
J Infect Dis
, vol.125
, pp. 427-440
-
-
Hunter, G.D.1
-
6
-
-
0020321767
-
Novel proteinaceous infectious particles cause scrapie
-
Prusiner SB (1982) Novel proteinaceous infectious particles cause scrapie. Science 216:136-144
-
(1982)
Science
, vol.216
, pp. 136-144
-
-
Prusiner, S.B.1
-
7
-
-
2442490085
-
Scrapie agent contains a hydrophobic protein
-
Prusiner SB, McKinley MP, Groth DF, Bowman KA, Mock NI, Cochran SP, Masiarz FR (1981) Scrapie agent contains a hydrophobic protein. Proc Natl Acad Sci USA 78:6675-6679
-
(1981)
Proc Natl Acad Sci USA
, vol.78
, pp. 6675-6679
-
-
Prusiner, S.B.1
McKinley, M.P.2
Groth, D.F.3
Bowman, K.A.4
Mock, N.I.5
Cochran, S.P.6
Masiarz, F.R.7
-
8
-
-
0021752457
-
Purification and structural studies of a major scrapie prion protein
-
Prusiner SB, Groth DF, Bolton DC, Kent SB, Hood LE (1984) Purification and structural studies of a major scrapie prion protein. Cell 38:127-134
-
(1984)
Cell
, vol.38
, pp. 127-134
-
-
Prusiner, S.B.1
Groth, D.F.2
Bolton, D.C.3
Kent, S.B.4
Hood, L.E.5
-
9
-
-
0011888936
-
Assignment of the human and mouse prion protein genes to homologous chromosomes
-
Sparkes RS, Simon M, Cohn VH, Fournier REK, Lem J, Klisak I, Heinzmann C, Blatt C, Lucero M, Mohandas T, DeArmond SJ, Westaway D, Prusiner SB, Weiner LP (1986) Assignment of the human and mouse prion protein genes to homologous chromosomes. Proc Natl Acad Sci USA 83:7358-7362
-
(1986)
Proc Natl Acad Sci USA
, vol.83
, pp. 7358-7362
-
-
Sparkes, R.S.1
Simon, M.2
Cohn, V.H.3
Fournier, R.E.K.4
Lem, J.5
Klisak, I.6
Heinzmann, C.7
Blatt, C.8
Lucero, M.9
Mohandas, T.10
DeArmond, S.J.11
Westaway, D.12
Prusiner, S.B.13
Weiner, L.P.14
-
10
-
-
0027332116
-
Conversion of α-helices into β-sheets features in the formation of the scrapie prion proteins
-
Pan K-M, Baldwin M, Nguyen J, Gasset M, Serban A, Groth D, Mehlhorn I, Huang Z, Fletterick RJ, Cohen FE, Prusiner SB (1993) Conversion of α-helices into β-sheets features in the formation of the scrapie prion proteins. Proc Natl Acad Sci USA 90:10962-10966
-
(1993)
Proc Natl Acad Sci USA
, vol.90
, pp. 10962-10966
-
-
Pan, K.-M.1
Baldwin, M.2
Nguyen, J.3
Gasset, M.4
Serban, A.5
Groth, D.6
Mehlhorn, I.7
Huang, Z.8
Fletterick, R.J.9
Cohen, F.E.10
Prusiner, S.B.11
-
11
-
-
0022005315
-
A cellular gene encodes scrapie PrP 27-30 protein
-
Oesch B, Westaway D, Wälchli M, McKinley MP, Kent SBH, Aebersold R, Barry RA, Tempst P, Teplow DB, Hood LE, Prusiner SB, Weissmann C (1985) A cellular gene encodes scrapie PrP 27-30 protein. Cell 40:735-746
-
(1985)
Cell
, vol.40
, pp. 735-746
-
-
Oesch, B.1
Westaway, D.2
Wälchli, M.3
McKinley, M.P.4
Kent, S.B.H.5
Aebersold, R.6
Barry, R.A.7
Tempst, P.8
Teplow, D.B.9
Hood, L.E.10
Prusiner, S.B.11
Weissmann, C.12
-
12
-
-
0040393220
-
Separation and properties of cellular and scrapie prion proteins
-
Meyer RK, McKinley MP, Bowman KA, Braunfeld MB, Barry RA, Prusiner SB (1986) Separation and properties of cellular and scrapie prion proteins. Proc Natl Acad Sci USA 83:2310-2314
-
(1986)
Proc Natl Acad Sci USA
, vol.83
, pp. 2310-2314
-
-
Meyer, R.K.1
McKinley, M.P.2
Bowman, K.A.3
Braunfeld, M.B.4
Barry, R.A.5
Prusiner, S.B.6
-
13
-
-
0025304678
-
Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells
-
Borchelt DR, Scott M, Taraboulos A, Stahl N, Prusiner SB (1990) Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells. J Cell Biol 110:743-752
-
(1990)
J Cell Biol
, vol.110
, pp. 743-752
-
-
Borchelt, D.R.1
Scott, M.2
Taraboulos, A.3
Stahl, N.4
Prusiner, S.B.5
-
14
-
-
0027534612
-
Structural analysis of the scrapie prion protein using mass spectrometry and amino acid sequencing
-
Stahl N, Baldwin MA, Teplow DB, Hood L, Gibson BW, Burlingame AL, Prusiner SB (1993) Structural analysis of the scrapie prion protein using mass spectrometry and amino acid sequencing. Biochemistry 32:1991-2002
-
(1993)
Biochemistry
, vol.32
, pp. 1991-2002
-
-
Stahl, N.1
Baldwin, M.A.2
Teplow, D.B.3
Hood, L.4
Gibson, B.W.5
Burlingame, A.L.6
Prusiner, S.B.7
-
15
-
-
0018360851
-
Creutzfeldt-Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering
-
Masters CL, Harris JO, Gajdusek DC, Gibbs CJ, Jr, Bernouilli C, Asher DM (1978) Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol 5:177-188
-
(1978)
Ann Neurol
, vol.5
, pp. 177-188
-
-
Masters, C.L.1
Harris, J.O.2
Gajdusek, D.C.3
Gibbs C.J., Jr.4
Bernouilli, C.5
Asher, D.M.6
-
17
-
-
0009585116
-
The epidemiology of Creutzfeldt-Jakob disease
-
Prusiner SB, Hadlow WJ, eds Academic Press, New York
-
Malmgren R, Kurland L, Mokri B, Kurtzke J (1979) The epidemiology of Creutzfeldt-Jakob disease, in Slow Transmissible Diseases of the Nervous System, Vol. 1 (Prusiner SB, Hadlow WJ, eds), pp 93-112. Academic Press, New York
-
(1979)
Slow Transmissible Diseases of the Nervous System
, vol.1
, pp. 93-112
-
-
Malmgren, R.1
Kurland, L.2
Mokri, B.3
Kurtzke, J.4
-
18
-
-
0025302625
-
Geographical distribution of cases of Creutzfeldt-Jakob disease in England and Wales 1970-1984
-
Cousens SN, Harries-Jones R, Knight R, Will RG, Smith PG, Matthews WB (1990) Geographical distribution of cases of Creutzfeldt-Jakob disease in England and Wales 1970-1984. J Neurol Neurosurg Psychiatry 53:459-465
-
(1990)
J Neurol Neurosurg Psychiatry
, vol.53
, pp. 459-465
-
-
Cousens, S.N.1
Harries-Jones, R.2
Knight, R.3
Will, R.G.4
Smith, P.G.5
Matthews, W.B.6
-
19
-
-
0023787829
-
Creutzfelt-Jakob disease in England and Wales, 1980-1984: A case-control study of potential risk factors
-
Harries-Jones R, Knight R, Will RG, Cousens S, Smith PG, Matthews WB (1988) Creutzfelt-Jakob disease in England and Wales, 1980-1984: a case-control study of potential risk factors. J Neurol Neurosurg Psychiatry 51:1113-1119
-
(1988)
J Neurol Neurosurg Psychiatry
, vol.51
, pp. 1113-1119
-
-
Harries-Jones, R.1
Knight, R.2
Will, R.G.3
Cousens, S.4
Smith, P.G.5
Matthews, W.B.6
-
20
-
-
84872473797
-
Advances in veterinary research
-
Gordon WS (1946) Advances in veterinary research. Vet Res 58:516-520
-
(1946)
Vet Res
, vol.58
, pp. 516-520
-
-
Gordon, W.S.1
-
21
-
-
0003782542
-
-
(Oppenheimer DR, ed.). Academic Press, New York
-
Parry HB (1983) Scrapie Disease in Sheep (Oppenheimer DR, ed.). Academic Press, New York
-
(1983)
Scrapie Disease in Sheep
-
-
Parry, H.B.1
-
22
-
-
0002977863
-
Scrapie: A transmissible and hereditary disease of sheep
-
Parry HB (1962) Scrapie: a transmissible and hereditary disease of sheep. Heredity 17:75-105
-
(1962)
Heredity
, vol.17
, pp. 75-105
-
-
Parry, H.B.1
-
24
-
-
0022973492
-
Creutzfeldt-Jakob disease: Clinical analysis of a consecutive series of 230 neuropathologically verified cases
-
Brown P, Cathala F, Castaigne P, Gajdusek DC (1986) Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol 20:597-602
-
(1986)
Ann Neurol
, vol.20
, pp. 597-602
-
-
Brown, P.1
Cathala, F.2
Castaigne, P.3
Gajdusek, D.C.4
-
25
-
-
0026606082
-
Atypical Creutzfeldt-Jakob disease in an American family with an insert mutation in the PRNP amyloid precursor gene
-
Brown P, Goldfarb LG, McCombie WR, Nieto A, Squillacote D, Sheremata W, Little BW, Godec MS, Gibbs CJ, Jr, Gajdusek DC (1992) Atypical Creutzfeldt-Jakob disease in an American family with an insert mutation in the PRNP amyloid precursor gene. Neurology 42:422-427
-
(1992)
Neurology
, vol.42
, pp. 422-427
-
-
Brown, P.1
Goldfarb, L.G.2
McCombie, W.R.3
Nieto, A.4
Squillacote, D.5
Sheremata, W.6
Little, B.W.7
Godec, M.S.8
Gibbs C.J., Jr.9
Gajdusek, D.C.10
-
26
-
-
0026690906
-
Fatal familial insomnia: A second kindred with mutation of prion protein gene at codon 178
-
Medori R, Montagna P, Tritschler HJ, LeBlanc A, Cortelli P, Tinuper P, Lugaresi E, Gambetti P (1992) Fatal familial insomnia: a second kindred with mutation of prion protein gene at codon 178. Neurology 42:669-670
-
(1992)
Neurology
, vol.42
, pp. 669-670
-
-
Medori, R.1
Montagna, P.2
Tritschler, H.J.3
LeBlanc, A.4
Cortelli, P.5
Tinuper, P.6
Lugaresi, E.7
Gambetti, P.8
-
27
-
-
0022518190
-
Familial myoclonic dementia masquerading as Creutzfeldt-Jakob disease
-
Little BW, Brown PW, Rodgers-Johnson P, Perl DP, Gajdusek DC (1986) Familial myoclonic dementia masquerading as Creutzfeldt-Jakob disease. Ann Neurol 20:231-239
-
(1986)
Ann Neurol
, vol.20
, pp. 231-239
-
-
Little, B.W.1
Brown, P.W.2
Rodgers-Johnson, P.3
Perl, D.P.4
Gajdusek, D.C.5
-
28
-
-
0026563279
-
Fatal familial insomnia: Clinical and pathological study of five new cases
-
Manetto V, Medori R, Cortelli P, Montagna P, Tinuper P, Baruzzi A, Rancurel G, Hauw J-J, Vanderhaeghen J-J, Mailleux P, Bugiani O, Tagliavini F, Bouras C, Ruzzuto N, Lugaresi E, Gambetti P (1992) Fatal familial insomnia: clinical and pathological study of five new cases. Neurology 42:312-319
-
(1992)
Neurology
, vol.42
, pp. 312-319
-
-
Manetto, V.1
Medori, R.2
Cortelli, P.3
Montagna, P.4
Tinuper, P.5
Baruzzi, A.6
Rancurel, G.7
Hauw, J.-J.8
Vanderhaeghen, J.-J.9
Mailleux, P.10
Bugiani, O.11
Tagliavini, F.12
Bouras, C.13
Ruzzuto, N.14
Lugaresi, E.15
Gambetti, P.16
-
29
-
-
0025681138
-
Spontaneous neurodegeneration in transgenic mice with mutant prion protein
-
Hsiao KK, Scott M, Foster D, Groth DF, DeArmond SJ, Prusiner SB (1990) Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science 250:1587-1590
-
(1990)
Science
, vol.250
, pp. 1587-1590
-
-
Hsiao, K.K.1
Scott, M.2
Foster, D.3
Groth, D.F.4
DeArmond, S.J.5
Prusiner, S.B.6
-
30
-
-
0025330687
-
Prion dementia without characteristic pathology
-
Collinge J, Owen F, Poulter H, Leach M, Crow T, Rosser M, Hardy J, Mullan H, Janota I, Lantos P (1990) Prion dementia without characteristic pathology. Lancet 336:7-9
-
(1990)
Lancet
, vol.336
, pp. 7-9
-
-
Collinge, J.1
Owen, F.2
Poulter, H.3
Leach, M.4
Crow, T.5
Rosser, M.6
Hardy, J.7
Mullan, H.8
Janota, I.9
Lantos, P.10
-
32
-
-
0141467070
-
Prion protein gene analysis and transmission studies of Creutzfeldt-Jakob disease
-
Prusiner SB, Collinge J, Powell J, Anderton B, eds Ellis Horwood, London
-
Tateishi J, Doh-ura K, Kitamoto T, Tranchant C, Steinmetz G, Warter JM, Boellaard JW (1992) Prion protein gene analysis and transmission studies of Creutzfeldt-Jakob disease, in Prion Diseases of Humans and Animals (Prusiner SB, Collinge J, Powell J, Anderton B, eds), pp 129-134. Ellis Horwood, London
-
(1992)
Prion Diseases of Humans and Animals
, pp. 129-134
-
-
Tateishi, J.1
Doh-Ura, K.2
Kitamoto, T.3
Tranchant, C.4
Steinmetz, G.5
Warter, J.M.6
Boellaard, J.W.7
-
33
-
-
0027535545
-
Real and imagined clinicopathological limits of 'prion dementia'
-
Brown P, Kaur P, Sulima MP, Goldfarb LG, Gibbs CJJ, Gajdusek DC (1993) Real and imagined clinicopathological limits of 'prion dementia'. Lancet 341:127-129
-
(1993)
Lancet
, vol.341
, pp. 127-129
-
-
Brown, P.1
Kaur, P.2
Sulima, M.P.3
Goldfarb, L.G.4
Gibbs, C.J.J.5
Gajdusek, D.C.6
-
34
-
-
0025910229
-
Molecular biology of prion diseases
-
Prusiner SB (1991) Molecular biology of prion diseases. Science 252:1515-1522
-
(1991)
Science
, vol.252
, pp. 1515-1522
-
-
Prusiner, S.B.1
-
35
-
-
0026487234
-
Prion protein immunocytochemistry helps to establish the true incidence of prion diseases
-
Lantos PL, McGill IS, Janota I, Doey LJ, Collinge J, Bruce MT, Whatley SA, Anderton BH, Clinton J, Roberts GW, Rossor MN (1992) Prion protein immunocytochemistry helps to establish the true incidence of prion diseases. Neurosci Lett 147:67-71
-
(1992)
Neurosci Lett
, vol.147
, pp. 67-71
-
-
Lantos, P.L.1
McGill, I.S.2
Janota, I.3
Doey, L.J.4
Collinge, J.5
Bruce, M.T.6
Whatley, S.A.7
Anderton, B.H.8
Clinton, J.9
Roberts, G.W.10
Rossor, M.N.11
-
36
-
-
0026650443
-
Inherited prion disease with 144 base pair gene insertion. 2. Clinical and pathological features
-
Collinge J, Brown J, Hardy J, Mullan M, Rossor MN, Baker H, Crow TJ, Lofthouse R, Poulter M, Ridley R, Owen F, Bennett C, Dunn G, Harding AE, Quinn N, Doshi B, Roberts GW, Honavar M, Janota I, Lantos PL (1992) Inherited prion disease with 144 base pair gene insertion. 2. Clinical and pathological features. Brain 115:687-710
-
(1992)
Brain
, vol.115
, pp. 687-710
-
-
Collinge, J.1
Brown, J.2
Hardy, J.3
Mullan, M.4
Rossor, M.N.5
Baker, H.6
Crow, T.J.7
Lofthouse, R.8
Poulter, M.9
Ridley, R.10
Owen, F.11
Bennett, C.12
Dunn, G.13
Harding, A.E.14
Quinn, N.15
Doshi, B.16
Roberts, G.W.17
Honavar, M.18
Janota, I.19
Lantos, P.L.20
more..
-
37
-
-
0001390048
-
Zwei eigenartige erkrankungen des zentralnervensystems nach art der spastischen pseudosklerose (Jakob)
-
Kirschbaum WR (1924) Zwei eigenartige Erkrankungen des Zentralnervensystems nach Art der spastischen Pseudosklerose (Jakob). Z Ges Neurol Psychiatr 92:175-220
-
(1924)
Z Ges Neurol Psychiatr
, vol.92
, pp. 175-220
-
-
Kirschbaum, W.R.1
-
38
-
-
0342929850
-
Weitere beiträge zum kapitel 'spastische pseudosklerose Jakobs'
-
Stender A (1930) Weitere Beiträge zum Kapitel 'Spastische Pseudosklerose Jakobs'. Z Neurol Psychiat 128:528-543
-
(1930)
Z Neurol Psychiat
, vol.128
, pp. 528-543
-
-
Stender, A.1
-
39
-
-
0000940311
-
Klinische und genealogische beobachtungen bei einem fall von spastischer pseudosklerose Jakobs
-
Meggendorfer F (1930) Klinische und genealogische Beobachtungen bei einem Fall von spastischer Pseudosklerose Jakobs. Z Ges Neurol Psychiatr 128:337-341
-
(1930)
Z Ges Neurol Psychiatr
, vol.128
, pp. 337-341
-
-
Meggendorfer, F.1
-
40
-
-
0342929806
-
Spastic pseudosclerosis (disseminated encephalomyelopathy; corticopal-lidospinal degeneration). Familial and nonfamilial incidence (a clinico-pathologic study)
-
Davison C, Rabiner AM (1940) Spastic pseudosclerosis (disseminated encephalomyelopathy; corticopal-lidospinal degeneration). Familial and nonfamilial incidence (a clinico-pathologic study). Arch Neurol Psychiatry 44:578-598
-
(1940)
Arch Neurol Psychiatry
, vol.44
, pp. 578-598
-
-
Davison, C.1
Rabiner, A.M.2
-
42
-
-
0343365104
-
Neuronal enzymatic failure in Creutzfeldt-Jakob disease. A familial study
-
Friede RL, DeJong RN (1964) Neuronal enzymatic failure in Creutzfeldt-Jakob disease. A familial study. Arch Neurol 10:181-195
-
(1964)
Arch Neurol
, vol.10
, pp. 181-195
-
-
Friede, R.L.1
DeJong, R.N.2
-
43
-
-
0017226461
-
Familial neurological disease associated with spongiform encephalopathy
-
Rosenthal NP, Keesey J, Crandall B, Brown WJ (1976) Familial neurological disease associated with spongiform encephalopathy. Arch Neurol 33:252-259
-
(1976)
Arch Neurol
, vol.33
, pp. 252-259
-
-
Rosenthal, N.P.1
Keesey, J.2
Crandall, B.3
Brown, W.J.4
-
44
-
-
0343800846
-
Familial Creutzfeldt-Jakob disease and other familial dementias: An inquiry into possible models of virus-induced familial diseases
-
Prusiner SB, Hadlow WJ, eds Academic Press, New York
-
Masters CL, Gajdusek DC, Gibbs CH, Jr, Bernouilli C, Asher DM (1979) Familial Creutzfeldt-Jakob disease and other familial dementias: an inquiry into possible models of virus-induced familial diseases, in Slow Transmissible Diseases of the Nervous System, Vol. 1 (Prusiner SB, Hadlow WJ, eds), pp 143-194. Academic Press, New York
-
(1979)
Slow Transmissible Diseases of the Nervous System
, vol.1
, pp. 143-194
-
-
Masters, C.L.1
Gajdusek, D.C.2
Gibbs Ch., Jr.3
Bernouilli, C.4
Asher, D.M.5
-
45
-
-
0022530549
-
Linkage of prion protein and scrapie incubation time genes
-
Carlson GA, Kingsbury DT, Goodman PA, Coleman S, Marshall ST, DeArmond SJ, Westaway D, Prusiner SB (1986) Linkage of prion protein and scrapie incubation time genes. Cell 46:503-511
-
(1986)
Cell
, vol.46
, pp. 503-511
-
-
Carlson, G.A.1
Kingsbury, D.T.2
Goodman, P.A.3
Coleman, S.4
Marshall, S.T.5
Dearmond, S.J.6
Westaway, D.7
Prusiner, S.B.8
-
46
-
-
0024519771
-
Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome
-
Hsiao K, Baker HF, Crow TJ, Poulter M, Owen F, Terwilliger JD, Westaway D, Ott J, Prusiner SB (1989) Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome. Nature 338:342-345
-
(1989)
Nature
, vol.338
, pp. 342-345
-
-
Hsiao, K.1
Baker, H.F.2
Crow, T.J.3
Poulter, M.4
Owen, F.5
Terwilliger, J.D.6
Westaway, D.7
Ott, J.8
Prusiner, S.B.9
-
47
-
-
0024473899
-
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, Kitamoto T, Sakaki Y (1989) 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 163:974-979
-
(1989)
Biochem Biophys Res Commun
, vol.163
, pp. 974-979
-
-
Doh-Ura, K.1
Tateishi, J.2
Sasaki, H.3
Kitamoto, T.4
Sakaki, Y.5
-
48
-
-
0024467653
-
Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker's syndrome
-
Goldgaber D, Goldfarb LG, Brown P, Asher DM, Brown WT, Lin S, Teener JW, Feinstone SM, Rubenstein R, Kascsak RJ, Boellaard JW, Gajdusek DC (1989) Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker's syndrome. Exp Neurol 106:204-206
-
(1989)
Exp Neurol
, vol.106
, pp. 204-206
-
-
Goldgaber, D.1
Goldfarb, L.G.2
Brown, P.3
Asher, D.M.4
Brown, W.T.5
Lin, S.6
Teener, J.W.7
Feinstone, S.M.8
Rubenstein, R.9
Kascsak, R.J.10
Boellaard, J.W.11
Gajdusek, D.C.12
-
49
-
-
0025906297
-
Prion protein mutation in family first reported by Gerstmann, Straussler, and Scheinker
-
Kretzschmar HA, Honold G, Seitelberger F, Feucht M, Wessely P, Mehraein P, Budka H (1991) Prion protein mutation in family first reported by Gerstmann, Straussler, and Scheinker. Lancet 337:1160
-
(1991)
Lancet
, vol.337
, pp. 1160
-
-
Kretzschmar, H.A.1
Honold, G.2
Seitelberger, F.3
Feucht, M.4
Wessely, P.5
Mehraein, P.6
Budka, H.7
-
50
-
-
0027730422
-
An Israeli family with Gerstmann-Straussler-Scheinker disease manifesting the codon 102 mutation in the prion protein gene
-
Goldhammer Y, Gabizon R, Meiner Z, Sadeh M (1993) An Israeli family with Gerstmann-Straussler-Scheinker disease manifesting the codon 102 mutation in the prion protein gene. Neur 43:2718-2719
-
(1993)
Neur
, vol.43
, pp. 2718-2719
-
-
Goldhammer, Y.1
Gabizon, R.2
Meiner, Z.3
Sadeh, M.4
-
51
-
-
0026636605
-
Inherited prion disease with 144 base pair gene insertion. 1. Genealogical and molecular studies
-
Poulter M, Baker HF, Frith CD, Leach M, Lofthouse R, Ridley RM, Shah T, Owen F, Collinge J, Brown G, Hardy J, Mullan MJ, Harding AE, Bennett C, Doshi R, Crow TJ (1992) Inherited prion disease with 144 base pair gene insertion. 1. Genealogical and molecular studies. Brain 115:675-685
-
(1992)
Brain
, vol.115
, pp. 675-685
-
-
Poulter, M.1
Baker, H.F.2
Frith, C.D.3
Leach, M.4
Lofthouse, R.5
Ridley, R.M.6
Shah, T.7
Owen, F.8
Collinge, J.9
Brown, G.10
Hardy, J.11
Mullan, M.J.12
Harding, A.E.13
Bennett, C.14
Doshi, R.15
Crow, T.J.16
-
52
-
-
0024530897
-
Insertion in prion protein gene in familial Creutzfeldt-Jakob disease
-
Owen F, Poulter M, Lofthouse R, Collinge J, Crow TJ, Risby D, Baker HF, Ridley RM, Hsiao K, Prusiner SB (1989) Insertion in prion protein gene in familial Creutzfeldt-Jakob disease. Lancet 1:51-52
-
(1989)
Lancet
, vol.1
, pp. 51-52
-
-
Owen, F.1
Poulter, M.2
Lofthouse, R.3
Collinge, J.4
Crow, T.J.5
Risby, D.6
Baker, H.F.7
Ridley, R.M.8
Hsiao, K.9
Prusiner, S.B.10
-
53
-
-
0025885702
-
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, Goldgaber D, Swergold GD, Wills PR, Cervenakova L, Baron H, Gibbs CJJ, Gajdusek DC (1991) Transmissible familial Creutzfeldt-Jakob disease associated with five, seven, and eight extra octapeptide coding repeats in the PRNP gene. Proc Natl Acad Sci USA 88:10926-10930
-
(1991)
Proc Natl Acad Sci USA
, vol.88
, pp. 10926-10930
-
-
Goldfarb, L.G.1
Brown, P.2
McCombie, W.R.3
Goldgaber, D.4
Swergold, G.D.5
Wills, P.R.6
Cervenakova, L.7
Baron, H.8
Gibbs, C.J.J.9
Gajdusek, D.C.10
-
54
-
-
0026513044
-
A dementing illness associated with a novel insertion in the prion protein gene
-
Owen F, Poulter M, Collinge J, Leach M, Lofthouse R, Crow TJ, Harding AE (1992) A dementing illness associated with a novel insertion in the prion protein gene. Mol Brain Res 13:155-157
-
(1992)
Mol Brain Res
, vol.13
, pp. 155-157
-
-
Owen, F.1
Poulter, M.2
Collinge, J.3
Leach, M.4
Lofthouse, R.5
Crow, T.J.6
Harding, A.E.7
-
55
-
-
0025010508
-
Deletion in prion protein gene in a Moroccan family
-
Laplanche J-L, Chatelain J, Launay J-M, Gazengel C, Vidaud M (1990) Deletion in prion protein gene in a Moroccan family. Nucleic Acids Res 18:6745
-
(1990)
Nucleic Acids Res
, vol.18
, pp. 6745
-
-
Laplanche, J.-L.1
Chatelain, J.2
Launay, J.-M.3
Gazengel, C.4
Vidaud, M.5
-
56
-
-
0026507882
-
Identification of heterogeneous PrP gene deletions in controls by detection of allele-specific heteroduplexes (DASH)
-
Vnencak-Jones CL, Phillips JA (1992) Identification of heterogeneous PrP gene deletions in controls by detection of allele-specific heteroduplexes (DASH). Am J Hum Genet 50:871-872
-
(1992)
Am J Hum Genet
, vol.50
, pp. 871-872
-
-
Vnencak-Jones, C.L.1
Phillips, J.A.2
-
57
-
-
0027270270
-
Deletions in the prion protein gene are not associated with CJD
-
Palmer MS, Mahal SP, Campbell TA, Hill AF, Sidle KCL, Laplanche J-L, Collinge J (1993) Deletions in the prion protein gene are not associated with CJD. Hum Molec Genet 2:541-544
-
(1993)
Hum Molec Genet
, vol.2
, pp. 541-544
-
-
Palmer, M.S.1
Mahal, S.P.2
Campbell, T.A.3
Hill, A.F.4
Sidle, K.C.L.5
Laplanche, J.-L.6
Collinge, J.7
-
58
-
-
0016389681
-
Creutzfeldt-Jakob disease: Focus among Libyan Jews in Israel
-
Kahana E, Milton A, Braham J, Sofer D (1974) Creutzfeldt-Jakob disease: focus among Libyan Jews in Israel. Science 183:90-91
-
(1974)
Science
, vol.183
, pp. 90-91
-
-
Kahana, E.1
Milton, A.2
Braham, J.3
Sofer, D.4
-
59
-
-
0025949664
-
Do Creutzfeldt-Jakob disease patients of Jewish Libyan origin have unique clinical features?
-
Kahana E, Zilber N, Abraham M (1991) Do Creutzfeldt-Jakob disease patients of Jewish Libyan origin have unique clinical features? Neurology 41:1390-1392
-
(1991)
Neurology
, vol.41
, pp. 1390-1392
-
-
Kahana, E.1
Zilber, N.2
Abraham, M.3
-
60
-
-
0024995430
-
Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia
-
Goldfarb LG, Mitrova E, Brown P, Toh BH, Gajdusek DC (1990) Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia. Lancet 336:514-515
-
(1990)
Lancet
, vol.336
, pp. 514-515
-
-
Goldfarb, L.G.1
Mitrova, E.2
Brown, P.3
Toh, B.H.4
Gajdusek, D.C.5
-
61
-
-
0025869213
-
Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease
-
Hsiao K, Meiner Z, Kahana E, Cass C, Kahana I, Avrahami D, Scarlato G, Abramsky O, Prusiner SB, Gabizon R (1991) Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. N Engl J Med 324:1091-1097
-
(1991)
N Engl J Med
, vol.324
, pp. 1091-1097
-
-
Hsiao, K.1
Meiner, Z.2
Kahana, E.3
Cass, C.4
Kahana, I.5
Avrahami, D.6
Scarlato, G.7
Abramsky, O.8
Prusiner, S.B.9
Gabizon, R.10
-
62
-
-
0025918142
-
Lys mutation: An analysis of 45 families
-
Lys mutation: an analysis of 45 families. Eur J Epidemiol 7:477-486
-
(1991)
Eur J Epidemiol
, vol.7
, pp. 477-486
-
-
Goldfarb, L.G.1
Brown, P.2
Mitrova, E.3
Cervenakova, L.4
Goldin, L.5
Korczyn, A.D.6
Chapman, J.7
Galvez, S.8
Cartier, L.9
Rubenstein, R.10
Gajdusek, D.C.11
-
64
-
-
0027404258
-
Inherited prion disease (PrP lysine 200) in Britain: Two case reports
-
Collinge J, Palmer MS, Campbell T, Sidle KCL, Carroll D, Harding A (1993) Inherited prion disease (PrP lysine 200) in Britain: two case reports. Br Med J 306:391-392
-
(1993)
Br Med J
, vol.306
, pp. 391-392
-
-
Collinge, J.1
Palmer, M.S.2
Campbell, T.3
Sidle, K.C.L.4
Carroll, D.5
Harding, A.6
-
65
-
-
85030205161
-
-
The Royal Society, Discussion Meeting on Molecular Biology of Prion Diseases, Sept 22-23, London
-
Kitamoto T (1993) Human prion diseases with PRNP polymorphisms. The Royal Society, Discussion Meeting on Molecular Biology of Prion Diseases, Sept 22-23, London
-
(1993)
Human Prion Diseases with PRNP Polymorphisms
-
-
Kitamoto, T.1
-
67
-
-
0026552106
-
Asn PRNP mutation in families of European origin
-
Asn PRNP mutation in families of European origin. Ann Neurol 31:274-281
-
(1992)
Ann Neurol
, vol.31
, pp. 274-281
-
-
Goldfarb, L.G.1
Brown, P.2
Haltia, M.3
Cathala, F.4
McCombie, W.R.5
Kovanen, J.6
Cervenakova, L.7
Goldin, L.8
Nieto, A.9
Godec, M.S.10
Asher, D.M.11
Gajdusek, D.C.12
-
68
-
-
0343365101
-
Fatal familial isomnia and one subtype of familial Creutzfeld-Jakob disease: Effect of a polymorphism on a pathogenic mutation in the prion protein
-
Petersen RB, Goldfarb L, Tabaton M, Brown P, LeBlanc A, Montagna P, Cortelli P, Monari L, Autilio-Gambetti L, Gajdusek DC, Lugaresi E, Gambetti P (1992) Fatal familial isomnia and one subtype of familial Creutzfeld-Jakob disease: effect of a polymorphism on a pathogenic mutation in the prion protein. FASEB J 7:A627
-
(1992)
FASEB J
, vol.7
-
-
Petersen, R.B.1
Goldfarb, L.2
Tabaton, M.3
Brown, P.4
LeBlanc, A.5
Montagna, P.6
Cortelli, P.7
Monari, L.8
Autilio-Gambetti, L.9
Gajdusek, D.C.10
Lugaresi, E.11
Gambetti, P.12
-
69
-
-
0026496257
-
Fatal familial insomnia and familial Creutzfeldt-Jakob disease: Disease phenotype determined by a DNA polymorphism
-
Goldfarb LG, Petersen RB, Tabaton M, Brown P, LeBlanc AC, Montagna P, Cortelli P, Julien J, Vital C, Pendelbury WW, Haltia M, Wills PR, Hauw JJ, McKeever PE, Monari L, Schrank B, Swergold GD, Autilio-Gambetti L, Gajdusek DC, Lugaresi E, Gambetti P (1992) Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism. Science 258:806-808
-
(1992)
Science
, vol.258
, pp. 806-808
-
-
Goldfarb, L.G.1
Petersen, R.B.2
Tabaton, M.3
Brown, P.4
LeBlanc, A.C.5
Montagna, P.6
Cortelli, P.7
Julien, J.8
Vital, C.9
Pendelbury, W.W.10
Haltia, M.11
Wills, P.R.12
Hauw, J.J.13
McKeever, P.E.14
Monari, L.15
Schrank, B.16
Swergold, G.D.17
Autilio-Gambetti, L.18
Gajdusek, D.C.19
Lugaresi, E.20
Gambetti, P.21
more..
-
70
-
-
0027378249
-
A new point mutation of the prion protein gene in familial and sporadic cases of Creutzfeldt-Jakob disease
-
Pocchiari M, Salvatore M, Cutruzzola F, Genuardi M, Travaglini Allocatelli C, Masullo C, Macchi G, Alema G, Galgani S, Xi YG, Petraroli P, Silvestrini MC, Brunori M (1993) A new point mutation of the prion protein gene in familial and sporadic cases of Creutzfeldt-Jakob disease. Ann Neurol 34:802-807
-
(1993)
Ann Neurol
, vol.34
, pp. 802-807
-
-
Pocchiari, M.1
Salvatore, M.2
Cutruzzola, F.3
Genuardi, M.4
Travaglini Allocatelli, C.5
Masullo, C.6
Macchi, G.7
Alema, G.8
Galgani, S.9
Xi, Y.G.10
Petraroli, P.11
Silvestrini, M.C.12
Brunori, M.13
-
71
-
-
0027425690
-
A new point mutation in the prion protein gene at codon 210 in Creutzfeldt-Jakob disease
-
Ripoll L, Laplanche J-L, Salzmann M, Jouvet A, Planques B, Dussaucy M, Chatelain J, Beaudry P, Launay J-M (1993) A new point mutation in the prion protein gene at codon 210 in Creutzfeldt-Jakob disease. Neurology 43:1934-1938
-
(1993)
Neurology
, vol.43
, pp. 1934-1938
-
-
Ripoll, L.1
Laplanche, J.-L.2
Salzmann, M.3
Jouvet, A.4
Planques, B.5
Dussaucy, M.6
Chatelain, J.7
Beaudry, P.8
Launay, J.-M.9
-
72
-
-
0025847141
-
A prion protein variant in a family with the telencephalic form of Gerstmann-Sträussler-Scheinker syndrome
-
Hsiao KK, Cass C, Schellenberg GD, Bird T, Devine-Gage E, Wisniewski H, Prusiner SB (1991) A prion protein variant in a family with the telencephalic form of Gerstmann-Sträussler-Scheinker syndrome. Neurology 41:681-684
-
(1991)
Neurology
, vol.41
, pp. 681-684
-
-
Hsiao, K.K.1
Cass, C.2
Schellenberg, G.D.3
Bird, T.4
Devine-Gage, E.5
Wisniewski, H.6
Prusiner, S.B.7
-
73
-
-
0026849947
-
Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles
-
Hsiao K, Dlouhy S, Farlow MR, Cass C, Da Costa M, Conneally M, Hodes ME, Ghetti B, Prusiner SB (1992) Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles. Nat Genet 1:68-71
-
(1992)
Nat Genet
, vol.1
, pp. 68-71
-
-
Hsiao, K.1
Dlouhy, S.2
Farlow, M.R.3
Cass, C.4
Da Costa, M.5
Conneally, M.6
Hodes, M.E.7
Ghetti, B.8
Prusiner, S.B.9
-
74
-
-
0027185917
-
Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Sträussler syndrome
-
Kitamoto T, Ohta M, Doh-ura K, Hitoshi S, Terao Y, Tateishi J (1993) Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Sträussler syndrome. Biochem Biophys Res Commun 191:709-714
-
(1993)
Biochem Biophys Res Commun
, vol.191
, pp. 709-714
-
-
Kitamoto, T.1
Ohta, M.2
Doh-Ura, K.3
Hitoshi, S.4
Terao, Y.5
Tateishi, J.6
-
75
-
-
0027236933
-
An amber mutation of prion protein in Gerstmann-Sträussler syndrome with mutant PrP plaques
-
Kitamoto T, Iizuka R, Tateishi J (1993) An amber mutation of prion protein in Gerstmann-Sträussler syndrome with mutant PrP plaques. Biochem Biophys Res Commun 192:525-531
-
(1993)
Biochem Biophys Res Commun
, vol.192
, pp. 525-531
-
-
Kitamoto, T.1
Iizuka, R.2
Tateishi, J.3
-
76
-
-
0024856333
-
Gerstmann-Sträussler-Scheinker disease. II. Neurofibrillary tangles and plaques with PrP-amyloid coexist in an affected family
-
Ghetti B, Tagliavini F, Masters CL, Beyreuther K, Giaccone G, Verga L, Farlo MR, Conneally PM, Dlouhy SR, Azzarelli B, Bugiani O (1989) Gerstmann-Sträussler-Scheinker disease. II. Neurofibrillary tangles and plaques with PrP-amyloid coexist in an affected family. Neurology 39:1453-1461
-
(1989)
Neurology
, vol.39
, pp. 1453-1461
-
-
Ghetti, B.1
Tagliavini, F.2
Masters, C.L.3
Beyreuther, K.4
Giaccone, G.5
Verga, L.6
Farlo, M.R.7
Conneally, P.M.8
Dlouhy, S.R.9
Azzarelli, B.10
Bugiani, O.11
-
77
-
-
0024324177
-
Familial dementia with PrP-positive amyloid plaques: A variant of Gerstmann-Sträussler syndrome
-
Nochlin D, Sumi SM, Bird TD, Snow AD, Leventhal CM, Beyreuther K, Masters CL (1989) Familial dementia with PrP-positive amyloid plaques: a variant of Gerstmann-Sträussler syndrome. Neurology 39:910-918
-
(1989)
Neurology
, vol.39
, pp. 910-918
-
-
Nochlin, D.1
Sumi, S.M.2
Bird, T.D.3
Snow, A.D.4
Leventhal, C.M.5
Beyreuther, K.6
Masters, C.L.7
-
78
-
-
0025013882
-
Neurofibrillary tangles of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease share antigenic determinants with those of Alzheimer disease
-
Giaccone G, Tagliavini F, Verga L, Frangione B, Farlow MR, Bugiani O, Ghetti B (1990) Neurofibrillary tangles of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease share antigenic determinants with those of Alzheimer disease. Brain Res 530:325-329
-
(1990)
Brain Res
, vol.530
, pp. 325-329
-
-
Giaccone, G.1
Tagliavini, F.2
Verga, L.3
Frangione, B.4
Farlow, M.R.5
Bugiani, O.6
Ghetti, B.7
-
79
-
-
0026033998
-
Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11-kd fragment of prion protein with an N-terminal glycine at codon 58
-
Tagliavini F, Prelli F, Ghisto J, Bugiani O, Serban D, Prusiner SB, Farlow MR, Ghetti B, Frangione B (1991) Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11-kd fragment of prion protein with an N-terminal glycine at codon 58. EMBO J 10:513-519
-
(1991)
EMBO J
, vol.10
, pp. 513-519
-
-
Tagliavini, F.1
Prelli, F.2
Ghisto, J.3
Bugiani, O.4
Serban, D.5
Prusiner, S.B.6
Farlow, M.R.7
Ghetti, B.8
Frangione, B.9
-
80
-
-
0026849545
-
Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene
-
Dlouhy SR, Hsiao K, Farlow MR, Foroud T, Conneally PM, Johnson P, Prusiner SB, Hodes ME, Ghetti B (1992) Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene. Nat Genet 1:64-67
-
(1992)
Nat Genet
, vol.1
, pp. 64-67
-
-
Dlouhy, S.R.1
Hsiao, K.2
Farlow, M.R.3
Foroud, T.4
Conneally, P.M.5
Johnson, P.6
Prusiner, S.B.7
Hodes, M.E.8
Ghetti, B.9
-
81
-
-
0343365099
-
A variant of Gerstmann-Sträussler-Scheinker disease with β-protein epitopes and dystrophic neurites in the peripheral regions of PrP-Immunoreactive amyloid plaques
-
Natvig JB, Forre O, Husby G, Husebekk A, Skogen B, Sletten K, Westermark P, eds, Kluwer Academic Publishers, Dordrecht
-
Ikeda S, Yanagisawa N, Allsop D, Glenner GG (1919) A variant of Gerstmann-Sträussler-Scheinker disease with β-protein epitopes and dystrophic neurites in the peripheral regions of PrP-Immunoreactive amyloid plaques, in Amyloid and Amyloidosis 1990 (Natvig JB, Forre O, Husby G, Husebekk A, Skogen B, Sletten K, Westermark P, eds), pp 737-740. Kluwer Academic Publishers, Dordrecht
-
(1919)
Amyloid and Amyloidosis 1990
, pp. 737-740
-
-
Ikeda, S.1
Yanagisawa, N.2
Allsop, D.3
Glenner, G.G.4
-
82
-
-
0026442278
-
Predicted α-helical regions of the prion protein when synthesized as peptides form amyloid
-
Gasset M, Baldwin MA, Lloyd D, Gabriel J-M, Holtzman DM, Cohen F, Fletterick R, Prusiner SB (1992) Predicted α-helical regions of the prion protein when synthesized as peptides form amyloid. Proc Natl Acad Sci USA 89:10940-10944
-
(1992)
Proc Natl Acad Sci USA
, vol.89
, pp. 10940-10944
-
-
Gasset, M.1
Baldwin, M.A.2
Lloyd, D.3
Gabriel, J.-M.4
Holtzman, D.M.5
Cohen, F.6
Fletterick, R.7
Prusiner, S.B.8
-
83
-
-
0027483615
-
Neurotoxicity of a prion protein fragment
-
Forloni G, Angeretti N, Chiesa R, Monzani E, Salmona M, Bugiani O, Tagliavini F (1993) Neurotoxicity of a prion protein fragment. Nature 362:543-546
-
(1993)
Nature
, vol.362
, pp. 543-546
-
-
Forloni, G.1
Angeretti, N.2
Chiesa, R.3
Monzani, E.4
Salmona, M.5
Bugiani, O.6
Tagliavini, F.7
-
84
-
-
0027270578
-
A kinetic model for amyloid formation in the prion diseases: Importance of seeding
-
Come JH, Fraser PE, Lansbury PT, Jr (1993) A kinetic model for amyloid formation in the prion diseases: importance of seeding. Proc Natl Acad Sci USA 90:5959-5963
-
(1993)
Proc Natl Acad Sci USA
, vol.90
, pp. 5959-5963
-
-
Come, J.H.1
Fraser, P.E.2
Lansbury P.T., Jr.3
-
85
-
-
0027159497
-
Synthetic peptides corresponding to different mutated regions of the amyloid gene in familial Creutzfeldt-Jakob disease show enhanced in vitro formation of morphologically different amyloid fibrils
-
Goldfarb LG, Brown P, Haltia M, Ghiso J, Frangione B, Gajdusek DC (1993) Synthetic peptides corresponding to different mutated regions of the amyloid gene in familial Creutzfeldt-Jakob disease show enhanced in vitro formation of morphologically different amyloid fibrils. Proc Natl Acad Sci USA 90:4451-4454
-
(1993)
Proc Natl Acad Sci USA
, vol.90
, pp. 4451-4454
-
-
Goldfarb, L.G.1
Brown, P.2
Haltia, M.3
Ghiso, J.4
Frangione, B.5
Gajdusek, D.C.6
-
86
-
-
0027491308
-
Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies
-
Prusiner SB, Groth D, Serban A, Koehler R, Foster D, Torchia M, Burton D, Yang S-L, DeArmond SJ (1993) Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies. Proc Natl Acad Sci USA 90:10608-10612
-
(1993)
Proc Natl Acad Sci USA
, vol.90
, pp. 10608-10612
-
-
Prusiner, S.B.1
Groth, D.2
Serban, A.3
Koehler, R.4
Foster, D.5
Torchia, M.6
Burton, D.7
Yang, S.-L.8
DeArmond, S.J.9
-
87
-
-
0027319326
-
Mice devoid of PrP are resistant to scrapie
-
Büeler H, Aguzzi A, Sailer A, Greiner R-A, Autenried P, Aguet M, Weissmann C (1993) Mice devoid of PrP are resistant to scrapie. Cell 73:1339-1347
-
(1993)
Cell
, vol.73
, pp. 1339-1347
-
-
Büeler, H.1
Aguzzi, A.2
Sailer, A.3
Greiner, R.-A.4
Autenried, P.5
Aguet, M.6
Weissmann, C.7
-
88
-
-
0028343093
-
No propagation of prions in mice devoid of PrP
-
Sailer A, Büeler H, Fischer M, Aguzzi A, Weissmann C (1994) No propagation of prions in mice devoid of PrP. Cell 77:967-968
-
(1994)
Cell
, vol.77
, pp. 967-968
-
-
Sailer, A.1
Büeler, H.2
Fischer, M.3
Aguzzi, A.4
Weissmann, C.5
-
89
-
-
0023663071
-
Scrapie prion protein contains a phosphatidylinositol glycolipid
-
Stahl N, Borchelt DR, Hsiao K, Prusiner SB (1987) Scrapie prion protein contains a phosphatidylinositol glycolipid. Cell 51:229-240
-
(1987)
Cell
, vol.51
, pp. 229-240
-
-
Stahl, N.1
Borchelt, D.R.2
Hsiao, K.3
Prusiner, S.B.4
-
90
-
-
0025991466
-
The scrapie-associated form of PrP is made from a cell surface precursor that is both protease-and phospholipase-sensitive
-
Caughey B, Raymond GJ (1991) The scrapie-associated form of PrP is made from a cell surface precursor that is both protease-and phospholipase-sensitive. J Biol Chem 266:18217-18223
-
(1991)
J Biol Chem
, vol.266
, pp. 18217-18223
-
-
Caughey, B.1
Raymond, G.J.2
-
91
-
-
0026775909
-
Evidence for synthesis of scrapie prion proteins in the endocytic pathway
-
Borchelt DR, Taraboulos A, Prusiner SB (1992) Evidence for synthesis of scrapie prion proteins in the endocytic pathway. J Biol Chem 267:16188-16199
-
(1992)
J Biol Chem
, vol.267
, pp. 16188-16199
-
-
Borchelt, D.R.1
Taraboulos, A.2
Prusiner, S.B.3
-
92
-
-
0028926204
-
Glycolipid-anchored proteins in neuroblastoma cells form detergent-resistant complexes without caveolin
-
Gorodinsky A, Harris DA (1995) Glycolipid-anchored proteins in neuroblastoma cells form detergent-resistant complexes without caveolin. J Cell Biol 129:619-627
-
(1995)
J Cell Biol
, vol.129
, pp. 619-627
-
-
Gorodinsky, A.1
Harris, D.A.2
-
93
-
-
0028966735
-
Cholesterol depletion and modification of COOH-terminal targeting sequence of the prion protein inhibit formation of the scrapie isoform
-
Taraboulos A, Scott M, Semenov A, Avrahami D, Laszlo L, Prusiner SB (1995) Cholesterol depletion and modification of COOH-terminal targeting sequence of the prion protein inhibit formation of the scrapie isoform. J Cell Biol 129:131-132
-
(1995)
J Cell Biol
, vol.129
, pp. 131-132
-
-
Taraboulos, A.1
Scott, M.2
Semenov, A.3
Avrahami, D.4
Laszlo, L.5
Prusiner, S.B.6
-
94
-
-
0026326210
-
Ultrastructural localization of scrapie prion proteins in cytoplasmic vesicles of infected cultured cells
-
McKinley MP, Taraboulos A, Kenaga L, Serban D, Stieber A, DeArmond SJ, Prusiner SB, Gonatas N (1991) Ultrastructural localization of scrapie prion proteins in cytoplasmic vesicles of infected cultured cells. Lab Invest 65:622-630
-
(1991)
Lab Invest
, vol.65
, pp. 622-630
-
-
McKinley, M.P.1
Taraboulos, A.2
Kenaga, L.3
Serban, D.4
Stieber, A.5
DeArmond, S.J.6
Prusiner, S.B.7
Gonatas, N.8
-
95
-
-
0029346911
-
The abnormal isoform of the prion protein accumulates in late-endosome-like organelles in scrapie-infected mouse brain
-
Arnold JE, Tipler C, Laszlo L, Hope J, Landon M, Mayer RJ (1995) The abnormal isoform of the prion protein accumulates in late-endosome-like organelles in scrapie-infected mouse brain. J Pathol 176:403-411
-
(1995)
J Pathol
, vol.176
, pp. 403-411
-
-
Arnold, J.E.1
Tipler, C.2
Laszlo, L.3
Hope, J.4
Landon, M.5
Mayer, R.J.6
-
96
-
-
0025244011
-
Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication
-
Prusiner SB, Scott M, Foster D, Pan K-M, Groth D, Mirenda C, Torchia M, Yang S-L, Serban D, Carlson GA, Hoppe PC, Westaway D, DeArmond SJ (1990) Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell 63:673-686
-
(1990)
Cell
, vol.63
, pp. 673-686
-
-
Prusiner, S.B.1
Scott, M.2
Foster, D.3
Pan, K.-M.4
Groth, D.5
Mirenda, C.6
Torchia, M.7
Yang, S.-L.8
Serban, D.9
Carlson, G.A.10
Hoppe, P.C.11
Westaway, D.12
DeArmond, S.J.13
-
97
-
-
0024820814
-
Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques
-
Scott M, Foster D, Mirenda C, Serban D, Coufal F, Wälchli M, Torchia M, Groth D, Carlson G, DeArmond SJ, Westaway D, Prusiner SB (1989) Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell 59:847-857
-
(1989)
Cell
, vol.59
, pp. 847-857
-
-
Scott, M.1
Foster, D.2
Mirenda, C.3
Serban, D.4
Coufal, F.5
Wälchli, M.6
Torchia, M.7
Groth, D.8
Carlson, G.9
DeArmond, S.J.10
Westaway, D.11
Prusiner, S.B.12
-
98
-
-
0028338441
-
Structural clues to the prion replication
-
Cohen FE, Pan K-M, Huang Z, Baldwin M, Fletterick RJ, Prusiner SB (1994) Structural clues to the prion replication. Science 264:530-531
-
(1994)
Science
, vol.264
, pp. 530-531
-
-
Cohen, F.E.1
Pan, K.-M.2
Huang, Z.3
Baldwin, M.4
Fletterick, R.J.5
Prusiner, S.B.6
-
99
-
-
0027353373
-
Prions and molecular chaperones
-
Liautard J-P (1993) Prions and molecular chaperones. Arch Virol 7 [Suppl.]:227-243
-
(1993)
Arch Virol
, vol.7
, Issue.SUPPL.
, pp. 227-243
-
-
Liautard, J.-P.1
-
100
-
-
0028288494
-
Expression and polyubiquitin and heat-shock protein 70 genes increases in the later stages of disease progression in scrapie-infected mouse brain
-
Kenward N, Hope J, Landon M, Mayer RJ (1994) Expression and polyubiquitin and heat-shock protein 70 genes increases in the later stages of disease progression in scrapie-infected mouse brain. J Neurochem 62:1870-1877
-
(1994)
J Neurochem
, vol.62
, pp. 1870-1877
-
-
Kenward, N.1
Hope, J.2
Landon, M.3
Mayer, R.J.4
-
101
-
-
0028958208
-
Scrapie prions selectively modify the stress response in neuroblastoma cells
-
Tatzelt J, Zuo J, Voellmy R, Scott M, Hartl U, Prusiner SB, Welch WJ (1995) Scrapie prions selectively modify the stress response in neuroblastoma cells. Proc Natl Acad Sci USA 92:2944-2948
-
(1995)
Proc Natl Acad Sci USA
, vol.92
, pp. 2944-2948
-
-
Tatzelt, J.1
Zuo, J.2
Voellmy, R.3
Scott, M.4
Hartl, U.5
Prusiner, S.B.6
Welch, W.J.7
-
102
-
-
0027086835
-
Chimeric prion protein expression in cultured cells and transgenic mice
-
Scott MR, Köhler R, Foster D, Prusiner SB (1992) Chimeric prion protein expression in cultured cells and transgenic mice. Protein Sci 1:986-997
-
(1992)
Protein Sci
, vol.1
, pp. 986-997
-
-
Scott, M.R.1
Köhler, R.2
Foster, D.3
Prusiner, S.B.4
-
103
-
-
0027229676
-
Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes
-
Scott M, Groth D, Foste D, Torchia M, Yang S-L, DeArmond SJ, Prusiner SB (1993) Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes. Cell 73:979-988
-
(1993)
Cell
, vol.73
, pp. 979-988
-
-
Scott, M.1
Groth, D.2
Foste, D.3
Torchia, M.4
Yang, S.-L.5
Dearmond, S.J.6
Prusiner, S.B.7
-
104
-
-
0028962532
-
Prion protein gene variation among primates
-
Schätzl HM, Da Costa M, Taylor L, Cohen FE, Prusiner SB (1995) Prion protein gene variation among primates. J Mol Biol 245:362-374
-
(1995)
J Mol Biol
, vol.245
, pp. 362-374
-
-
Schätzl, H.M.1
Da Costa, M.2
Taylor, L.3
Cohen, F.E.4
Prusiner, S.B.5
-
105
-
-
0028102794
-
Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein
-
Telling GC, Scott M, Hsiao KK, Foster D, Yang S-L, Torchia M, Sidle KCL, Collinge J, DeArmond SJ, Prusiner SB (1994) Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. Proc Natl Acad Sci USA 91:9936-9940
-
(1994)
Proc Natl Acad Sci USA
, vol.91
, pp. 9936-9940
-
-
Telling, G.C.1
Scott, M.2
Hsiao, K.K.3
Foster, D.4
Yang, S.-L.5
Torchia, M.6
Sidle, K.C.L.7
Collinge, J.8
DeArmond, S.J.9
Prusiner, S.B.10
-
106
-
-
0026600865
-
Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein
-
Büeler H, Fischer M, Lang Y, Bluethmann H, Lipp H-P, DeArmond SJ, Prusiner SB, Aguet M, Weissmann C (1992) Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature 356:577-582
-
(1992)
Nature
, vol.356
, pp. 577-582
-
-
Büeler, H.1
Fischer, M.2
Lang, Y.3
Bluethmann, H.4
Lipp, H.-P.5
DeArmond, S.J.6
Prusiner, S.B.7
Aguet, M.8
Weissmann, C.9
-
107
-
-
0028882424
-
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, Gabizon R, Torchia M, Cohen FE, DeArmond SJ, Prusiner SB (1995) Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell 83:79-90
-
(1995)
Cell
, vol.83
, pp. 79-90
-
-
Telling, G.C.1
Scott, M.2
Mastrianni, J.3
Gabizon, R.4
Torchia, M.5
Cohen, F.E.6
DeArmond, S.J.7
Prusiner, S.B.8
-
108
-
-
0025165213
-
Three hamster species with different scrapie incubation times and neuropathological features encode distinct prion proteins
-
Lowenstein DH, Butler DA, Westaway D, McKinley MP, DeArmond SJ, Prusiner SB (1990) Three hamster species with different scrapie incubation times and neuropathological features encode distinct prion proteins. Mol Cell Biol 10:1153-1163
-
(1990)
Mol Cell Biol
, vol.10
, pp. 1153-1163
-
-
Lowenstein, D.H.1
Butler, D.A.2
Westaway, D.3
McKinley, M.P.4
DeArmond, S.J.5
Prusiner, S.B.6
-
109
-
-
0028339962
-
Proposed three-dimensional structure for the cellular prion protein
-
Huang Z, Gabriel J-M, Baldwin MA, Fletterick RJ, Prusiner SB, Cohen FE (1994) Proposed three-dimensional structure for the cellular prion protein. Proc Natl Acad Sci USA 91:7139-7143
-
(1994)
Proc Natl Acad Sci USA
, vol.91
, pp. 7139-7143
-
-
Huang, Z.1
Gabriel, J.-M.2
Baldwin, M.A.3
Fletterick, R.J.4
Prusiner, S.B.5
Cohen, F.E.6
-
110
-
-
0026702071
-
Attempts to convert the cellular prion protein into the scrapie isoform in cell-free systems
-
Raeber AJ, Borchelt DR, Scott M, Prusiner SB (1992) Attempts to convert the cellular prion protein into the scrapie isoform in cell-free systems. J Virol 66:6155-6163
-
(1992)
J Virol
, vol.66
, pp. 6155-6163
-
-
Raeber, A.J.1
Borchelt, D.R.2
Scott, M.3
Prusiner, S.B.4
-
111
-
-
0028925377
-
Prion protein peptides induce α-helix to β-sheet conformational transitions
-
Nguyen J, Baldwin MA, Cohen FE, Prusiner SB (1995) Prion protein peptides induce α-helix to β-sheet conformational transitions. Biochemistry 34:4186-4192
-
(1995)
Biochemistry
, vol.34
, pp. 4186-4192
-
-
Nguyen, J.1
Baldwin, M.A.2
Cohen, F.E.3
Prusiner, S.B.4
-
112
-
-
0027956109
-
Cell-free formation of protease-resistant prion protein
-
Kocisko DA, Come JH, Priola SA, Chesebro B, Raymond GJ, Lansbury PT, Jr, Caughey B (1994) Cell-free formation of protease-resistant prion protein. Nature 370:471-474
-
(1994)
Nature
, vol.370
, pp. 471-474
-
-
Kocisko, D.A.1
Come, J.H.2
Priola, S.A.3
Chesebro, B.4
Raymond, G.J.5
Lansbury P.T., Jr.6
Caughey, B.7
-
113
-
-
0029116625
-
Conformational transitions in peptides containing two putative α-helices of the prion protein
-
Zhang H, Kaneko K, Nguyen JT, Livshits TL, Baldwin MA, Cohen FE, James TL, Prusiner SB (1995) Conformational transitions in peptides containing two putative α-helices of the prion protein. J Mol Biol 250:514-526
-
(1995)
J Mol Biol
, vol.250
, pp. 514-526
-
-
Zhang, H.1
Kaneko, K.2
Nguyen, J.T.3
Livshits, T.L.4
Baldwin, M.A.5
Cohen, F.E.6
James, T.L.7
Prusiner, S.B.8
-
114
-
-
0029149975
-
X-ray diffraction of scrapie prion rods and PrP peptides
-
Nguyen JT, Inouye H, Baldwin MA, Fletterick RH, Cohen FE, Prusiner SB, Kirschner DA (1995) X-ray diffraction of scrapie prion rods and PrP peptides. J Mol Biol 252:412-422
-
(1995)
J Mol Biol
, vol.252
, pp. 412-422
-
-
Nguyen, J.T.1
Inouye, H.2
Baldwin, M.A.3
Fletterick, R.H.4
Cohen, F.E.5
Prusiner, S.B.6
Kirschner, D.A.7
-
115
-
-
0028867086
-
Prion protein (PrP) synthetic peptides induce cellular PrP to acquire properties of the scrapie isoform
-
Kaneko K, Peretz D, Pan K-M, Blochberger T, Gabizon R, Griffith OH, Cohen FE, Baldwin MA, Prusiner SB (1995) Prion protein (PrP) synthetic peptides induce cellular PrP to acquire properties of the scrapie isoform. Proc Natl Acad Sci USA 32:11160-11164
-
(1995)
Proc Natl Acad Sci USA
, vol.32
, pp. 11160-11164
-
-
Kaneko, K.1
Peretz, D.2
Pan, K.-M.3
Blochberger, T.4
Gabizon, R.5
Griffith, O.H.6
Cohen, F.E.7
Baldwin, M.A.8
Prusiner, S.B.9
-
116
-
-
0029066886
-
Species specificity in the cell-free conversion of prion protein to protease-resistant forms: A model for the scrapie species barrier
-
Kocisko DA, Priola SA, Raymond GJ, Chesebro B, Lansbury PT, Jr, Caughey B (1995) Species specificity in the cell-free conversion of prion protein to protease-resistant forms: a model for the scrapie species barrier. Proc Natl Acad Sci USA 92:3923-3927
-
(1995)
Proc Natl Acad Sci USA
, vol.92
, pp. 3923-3927
-
-
Kocisko, D.A.1
Priola, S.A.2
Raymond, G.J.3
Chesebro, B.4
Lansbury P.T., Jr.5
Caughey, B.6
-
117
-
-
0028997297
-
Non-genetic propagation of strain-specific properties of scrapie prion protein
-
Bessen RA, Kocisko DA, Raymond GJ, Nandan S, Lansbury PT, Caughey B (1995) Non-genetic propagation of strain-specific properties of scrapie prion protein. Nature 375:698-700
-
(1995)
Nature
, vol.375
, pp. 698-700
-
-
Bessen, R.A.1
Kocisko, D.A.2
Raymond, G.J.3
Nandan, S.4
Lansbury, P.T.5
Caughey, B.6
-
118
-
-
0015177637
-
Host-genotype and agent effects in scrapie incubation: Change in allelic interaction with different strains of agent
-
Dickinson AG, Meikle VMH (1971) Host-genotype and agent effects in scrapie incubation: change in allelic interaction with different strains of agent. Mol Gen Genet 112:73-79
-
(1971)
Mol Gen Genet
, vol.112
, pp. 73-79
-
-
Dickinson, A.G.1
Meikle, V.M.H.2
-
119
-
-
0028276015
-
Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice
-
Carlson GA, Ebeling C, Yang S-L, Telling G, Torchia M, Groth D, Westaway D, DeArmond SJ, Prusiner SB (1994) Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice. Proc Natl Acad Svi USA 91:5690-5694
-
(1994)
Proc Natl Acad Svi USA
, vol.91
, pp. 5690-5694
-
-
Carlson, G.A.1
Ebeling, C.2
Yang, S.-L.3
Telling, G.4
Torchia, M.5
Groth, D.6
Westaway, D.7
DeArmond, S.J.8
Prusiner, S.B.9
-
121
-
-
0023888782
-
Scrapie prion liposomes and rods exhibit target size of 55,000 Da
-
Bellinger-Kawahara CG, Kempner E, Groth DF, Gabizon R, Prusiner SB (1988) Scrapie prion liposomes and rods exhibit target size of 55,000 Da. Virology 164:537-541
-
(1988)
Virology
, vol.164
, pp. 537-541
-
-
Bellinger-Kawahara, C.G.1
Kempner, E.2
Groth, D.F.3
Gabizon, R.4
Prusiner, S.B.5
-
122
-
-
0027520888
-
Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cells
-
Rogers M, Yehiely F, Scott M, Prusiner SB (1993) Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cells. Proc Natl Acad Sci USA 90:3182-3186
-
(1993)
Proc Natl Acad Sci USA
, vol.90
, pp. 3182-3186
-
-
Rogers, M.1
Yehiely, F.2
Scott, M.3
Prusiner, S.B.4
-
123
-
-
0028878943
-
Inherited prion diseases and transmission to rodents
-
Tateishi J, Kitamoto T (1995) Inherited prion diseases and transmission to rodents. Brain Pathol 5:53-59
-
(1995)
Brain Pathol
, vol.5
, pp. 53-59
-
-
Tateishi, J.1
Kitamoto, T.2
-
124
-
-
0024211952
-
Transmissible and non-transmissible amyloidoses: Autocatalytic post-translational conversion of host precursor proteins to β-pleated sheet configurations
-
Gajdusek DC (1988) Transmissible and non-transmissible amyloidoses: autocatalytic post-translational conversion of host precursor proteins to β-pleated sheet configurations. J Neuroimmunol 20:95-110
-
(1988)
J Neuroimmunol
, vol.20
, pp. 95-110
-
-
Gajdusek, D.C.1
-
125
-
-
0000857618
-
Subacute spongiform encephalopathies: Transmissible cerebral amyloidoses caused by unconventional viruses
-
(Fields BN, Knipe DM, Chanock RM, Hirsh MS, Melnick JL, Monath TP, Roizman B, eds), Raven Press, New York
-
Gajdusek DC (1990) Subacute spongiform encephalopathies: transmissible cerebral amyloidoses caused by unconventional viruses, in Virology, 2nd Ed. (Fields BN, Knipe DM, Chanock RM, Hirsh MS, Melnick JL, Monath TP, Roizman B, eds), pp 2289-2324. Raven Press, New York
-
(1990)
Virology, 2nd Ed.
, pp. 2289-2324
-
-
Gajdusek, D.C.1
-
126
-
-
0343365087
-
Brain amyloidoses-precursor proteins and the amyloids of transmissible and nontransmissible dementias: Scrapie-kuru-CJD viruses as infectious polypeptides or amyloid-enhancing vector
-
Goldstein A, ed., Plenum Publishing Corporation, New York
-
Gajdusek DC, Gibbs CJ, Jr (1990) Brain amyloidoses-precursor proteins and the amyloids of transmissible and nontransmissible dementias: scrapie-kuru-CJD viruses as infectious polypeptides or amyloid-enhancing vector, in Biomedical Advances in Aging (Goldstein A, ed.), pp 3-24. Plenum Publishing Corporation, New York
-
(1990)
Biomedical Advances in Aging
, pp. 3-24
-
-
Gajdusek, D.C.1
Gibbs C.J., Jr.2
-
127
-
-
0027195933
-
Seeding "one-dimensional crystallization" of amyloid: A pathogenic mechanism in Alzheimer's disease and scrapie?
-
Jarrett JT, Lansbury PT, Jr (1993) Seeding "one-dimensional crystallization" of amyloid: a pathogenic mechanism in Alzheimer's disease and scrapie? Cell 73:1055-1058
-
(1993)
Cell
, vol.73
, pp. 1055-1058
-
-
Jarrett, J.T.1
Lansbury P.T., Jr.2
-
128
-
-
0027182522
-
Conformational transitions, dissociation, and unfolding of scrapie amyloid (prion) protein
-
Safar J, Roller PP, Gajdusek DC, Gibbs CJ, Jr (1993) Conformational transitions, dissociation, and unfolding of scrapie amyloid (prion) protein. J Biol Chem 268:20276-20284
-
(1993)
J Biol Chem
, vol.268
, pp. 20276-20284
-
-
Safar, J.1
Roller, P.P.2
Gajdusek, D.C.3
Gibbs C.J., Jr.4
-
129
-
-
0016329537
-
Beta-pleated sheet fibrils - A comparison of native amyloid with synthetic protein fibrils
-
Glenner GG, Eanes ED, Bladen HA, Linke RP, Termine JD (1974) Beta-pleated sheet fibrils - a comparison of native amyloid with synthetic protein fibrils. J Histochem Cytochem 22:1141-1158
-
(1974)
J Histochem Cytochem
, vol.22
, pp. 1141-1158
-
-
Glenner, G.G.1
Eanes, E.D.2
Bladen, H.A.3
Linke, R.P.4
Termine, J.D.5
-
130
-
-
0019153066
-
Amyloid deposits and amyloidosis
-
Glenner GG (1980) Amyloid deposits and amyloidosis. N Engl J Med 302:1283-1292
-
(1980)
N Engl J Med
, vol.302
, pp. 1283-1292
-
-
Glenner, G.G.1
-
131
-
-
0026062496
-
Scrapie prion rod formation in vitro requires both detergent extraction and limited proteolysis
-
McKinley MP, Meyer R, Kenaga L, Rahbar F, Cotter R, Serban A, Prusiner SB (1991) Scrapie prion rod formation in vitro requires both detergent extraction and limited proteolysis. J Virol 65:1440-1449
-
(1991)
J Virol
, vol.65
, pp. 1440-1449
-
-
McKinley, M.P.1
Meyer, R.2
Kenaga, L.3
Rahbar, F.4
Cotter, R.5
Serban, A.6
Prusiner, S.B.7
-
132
-
-
0343364948
-
Purified prion proteins and scrapie infectivity copartition into liposomes
-
Gabizon R, McKinley MP, Prusiner SB (1987) Purified prion proteins and scrapie infectivity copartition into liposomes. Proc Natl Acad Sci USA 84:4017-4021
-
(1987)
Proc Natl Acad Sci USA
, vol.84
, pp. 4017-4021
-
-
Gabizon, R.1
McKinley, M.P.2
Prusiner, S.B.3
-
133
-
-
0027373649
-
Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease
-
Gabizon R, Rosenmann H, Meiner Z, Kahana I, Kahana E, Shugart Y, Ott J, Prusiner SB (1993) Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease. Am J Hum Genet 33:828-835
-
(1993)
Am J Hum Genet
, vol.33
, pp. 828-835
-
-
Gabizon, R.1
Rosenmann, H.2
Meiner, Z.3
Kahana, I.4
Kahana, E.5
Shugart, Y.6
Ott, J.7
Prusiner, S.B.8
-
134
-
-
0028782020
-
Human prion diseases with variant prion protein
-
Kitamoto T, Tateishi J (1994) Human prion diseases with variant prion protein. Phil Trans R Soc Lond B 343:391-398
-
(1994)
Phil Trans R Soc Lond B
, vol.343
, pp. 391-398
-
-
Kitamoto, T.1
Tateishi, J.2
-
135
-
-
0022477981
-
Molecular cloning of a human prion protein cDNA
-
Kretzschmar HA, Stowring LE, Westaway D, Stubblebine WH, Prusiner SB, DeArmond SJ (1986) Molecular cloning of a human prion protein cDNA. DNA 5:315-324
-
(1986)
DNA
, vol.5
, pp. 315-324
-
-
Kretzschmar, H.A.1
Stowring, L.E.2
Westaway, D.3
Stubblebine, W.H.4
Prusiner, S.B.5
DeArmond, S.J.6
-
137
-
-
0025820942
-
Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease
-
Palmer MS, Dryden AJ, Hughes JT, Collinge J (1991) Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature 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
-
138
-
-
0028287867
-
Detecting prion protein gene-mutations by denaturing gradient gel-electrophoresis
-
Fink JK, Peacock ML, Warren JT, Roses AD, Prusiner SB (1994) Detecting prion protein gene-mutations by denaturing gradient gel-electrophoresis. Hum Mutat 4:42-50
-
(1994)
Hum Mutat
, vol.4
, pp. 42-50
-
-
Fink, J.K.1
Peacock, M.L.2
Warren, J.T.3
Roses, A.D.4
Prusiner, S.B.5
-
139
-
-
0025055393
-
An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease
-
Owen F, Poulter M, Shah T, Collinge J, Lofthouse R, Baker H, Ridley R, McVey J, Crow T (1990) An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease. Mol Brain Res 7:273-276
-
(1990)
Mol Brain Res
, vol.7
, pp. 273-276
-
-
Owen, F.1
Poulter, M.2
Shah, T.3
Collinge, J.4
Lofthouse, R.5
Baker, H.6
Ridley, R.7
McVey, J.8
Crow, T.9
-
140
-
-
0024474822
-
Diagnosis of Gerstmann-Sträussler syndrome in familial dementia with prion protein gene analysis
-
Collinge J, Harding AE, Owen F, Poulter M, Lofthouse R, Boughey AM, Shah T, Crow TJ (1989) Diagnosis of Gerstmann-Sträussler syndrome in familial dementia with prion protein gene analysis. Lancet 2:15-17
-
(1989)
Lancet
, vol.2
, pp. 15-17
-
-
Collinge, J.1
Harding, A.E.2
Owen, F.3
Poulter, M.4
Lofthouse, R.5
Boughey, A.M.6
Shah, T.7
Crow, T.J.8
-
141
-
-
12644277724
-
Mutations in the prion gene in human transmissible dementia
-
Medical Research Council, London, Dec 14, 1990 (Abstr)
-
Crow TJ, Collinge J, Ridley RM, Baker HF, Lofthouse R, Owen F, Harding AE (1990) Mutations in the prion gene in human transmissible dementia. Seminar on Molecular Approaches to Research in Spongiform Encephalopathies in Man, Medical Research Council, London, Dec 14, 1990 (Abstr)
-
(1990)
Seminar on Molecular Approaches to Research in Spongiform Encephalopathies in Man
-
-
Crow, T.J.1
Collinge, J.2
Ridley, R.M.3
Baker, H.F.4
Lofthouse, R.5
Owen, F.6
Harding, A.E.7
-
142
-
-
0025339222
-
Creutzfeldt-Jakob disease and kuru patients lack a mutation consistently found in the Gerstmann-Sträussler-Scheinker syndrome
-
Goldfarb LG, Brown P, Goldgaber D, Asher DM, Rubenstein R, Brown WT, Piccardo P, Kascsak RJ, Boellaard JW, Gajdusek DC (1990) Creutzfeldt-Jakob disease and kuru patients lack a mutation consistently found in the Gerstmann-Sträussler-Scheinker syndrome. Exp Neurol 108:247-250
-
(1990)
Exp Neurol
, vol.108
, pp. 247-250
-
-
Goldfarb, L.G.1
Brown, P.2
Goldgaber, D.3
Asher, D.M.4
Rubenstein, R.5
Brown, W.T.6
Piccardo, P.7
Kascsak, R.J.8
Boellaard, J.W.9
Gajdusek, D.C.10
-
143
-
-
0025280532
-
Identical mutation in unrelated patients with Creutzfeldt-Jakob disease
-
Goldfarb L, Brown P, Goldgaber D, Garruto R, Yanaghiara R, Asher D, Gajdusek DC (1990) Identical mutation in unrelated patients with Creutzfeldt-Jakob disease. Lancet 336:174-175
-
(1990)
Lancet
, vol.336
, pp. 174-175
-
-
Goldfarb, L.1
Brown, P.2
Goldgaber, D.3
Garruto, R.4
Yanaghiara, R.5
Asher, D.6
Gajdusek, D.C.7
-
144
-
-
0000672037
-
A prion protein amino acid substitution in ataxic Gerstmann-Sträussler syndrome
-
Hsiao K, Doh-ura K, Kitamoto T, Tateishi J, Prusiner SB (1989) A prion protein amino acid substitution in ataxic Gerstmann-Sträussler syndrome. Ann Neurol 26:137
-
(1989)
Ann Neurol
, vol.26
, pp. 137
-
-
Hsiao, K.1
Doh-Ura, K.2
Kitamoto, T.3
Tateishi, J.4
Prusiner, S.B.5
-
145
-
-
0025641602
-
Inherited human prion diseases
-
Hsiao K, Prusiner SB (1990) Inherited human prion diseases. Neurology 40:1820-1827
-
(1990)
Neurology
, vol.40
, pp. 1820-1827
-
-
Hsiao, K.1
Prusiner, S.B.2
-
146
-
-
0025119522
-
Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-Sträussler-Scheinker syndrome
-
Tateishi J, Kitamoto T, Doh-ura K, Sakaki Y, Steinmetz G, Tranchant C, Warter JM, Heldt N (1990) Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-Sträussler-Scheinker syndrome. Neurology 40:1578-1581
-
(1990)
Neurology
, vol.40
, pp. 1578-1581
-
-
Tateishi, J.1
Kitamoto, T.2
Doh-Ura, K.3
Sakaki, Y.4
Steinmetz, G.5
Tranchant, C.6
Warter, J.M.7
Heldt, N.8
-
147
-
-
0024992359
-
Mutation in codon 200 of scrapie amyloid precursor gene linked to Creutzfeldt-Jakob disease in Sephardic Jews of Libyan and non-Libyan origin
-
Goldfarb L, Korczyn A, Brown P, Chapman J, Gajdusek DC (1990) Mutation in codon 200 of scrapie amyloid precursor gene linked to Creutzfeldt-Jakob disease in Sephardic Jews of Libyan and non-Libyan origin. Lancet 336:637-638
-
(1990)
Lancet
, vol.336
, pp. 637-638
-
-
Goldfarb, L.1
Korczyn, A.2
Brown, P.3
Chapman, J.4
Gajdusek, D.C.5
-
148
-
-
0005828275
-
Prion protein gene mutation in Libyan Jews with Creutzfeldt-Jakob disease
-
Gabizon R, Meiner Z, Cass C, Kahana E, Kahana I, Avrahami D, Abramsky O, Scarlato G, Prusiner SB, Hsiao KK (1991) Prion protein gene mutation in Libyan Jews with Creutzfeldt-Jakob disease. Neurology 41:160
-
(1991)
Neurology
, vol.41
, pp. 160
-
-
Gabizon, R.1
Meiner, Z.2
Cass, C.3
Kahana, E.4
Kahana, I.5
Avrahami, D.6
Abramsky, O.7
Scarlato, G.8
Prusiner, S.B.9
Hsiao, K.K.10
-
149
-
-
0026061028
-
New mutation in scrapie amyloid precursor gene (at codon 178) in Finnish Creutzfeldt-Jakob kindred
-
Goldfarb LG, Haltia M, Brown P, Nieto A, Kovanen J, McCombie WR, Trapp S, Gajdusek DC (1991) New mutation in scrapie amyloid precursor gene (at codon 178) in Finnish Creutzfeldt-Jakob kindred. Lancet 337:425
-
(1991)
Lancet
, vol.337
, pp. 425
-
-
Goldfarb, L.G.1
Haltia, M.2
Brown, P.3
Nieto, A.4
Kovanen, J.5
McCombie, W.R.6
Trapp, S.7
Gajdusek, D.C.8
-
150
-
-
0007566581
-
Identification of a new mutation of the prion protein gene at codon 208 in a patient with Creutzfeldt-Jakob disease
-
Mastrianni JA, Iannicola C, Myers R, Prusiner SB (1995) Identification of a new mutation of the prion protein gene at codon 208 in a patient with Creutzfeldt-Jakob disease (Abstr). Neurology 45 [Suppl.]:201
-
(1995)
Neurology
, vol.45
, Issue.SUPPL.
, pp. 201
-
-
Mastrianni, J.A.1
Iannicola, C.2
Myers, R.3
Prusiner, S.B.4
|