-
1
-
-
0000049491
-
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
-
4
-
-
0018936053
-
Amyx HL, Bacote A, et al
-
Gibbs CJ, Jr., Amyx HL, Bacote A, et al. Oral transmission of kuru, Creutzfeldt-Jakob disease and scrapie to nonhuman primates. J Infect Dis 1980; 142:205-208.
-
(1980)
Oral Transmission of Kuru, Creutzfeldt-Jakob Disease and Scrapie to Nonhuman Primates. J Infect Dis
, vol.142
, pp. 205-208
-
-
Gibbs, C.J.1
-
5
-
-
49749206702
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
41
-
-
0007813199
-
Indiana kindred of Gerstmann-Straussler-Scheinker disease: Neurofibrillary tangles and neuntes of plaques with PrP amyloid share antigenic determinants with those of Alzheimer's disease
-
Giaccone G, Tagliavini F, Verga L, et al. Indiana kindred of Gerstmann-Straussler-Scheinker disease: neurofibrillary tangles and neuntes of plaques with PrP amyloid share antigenic determinants with those of Alzheimer's disease. In: Iqbal K, McLachlan D, Winblad B, et al, eds. Alzheimer's disease: basic mechanisms, diagnosis and therapeutic strategies. Chichester, UK: John Wiley & Sons, 1991:207-211.
-
(1991)
In: Iqbal K, McLachlan D, Winblad B, Et Al, Eds. Alzheimer's Disease: Basic Mechanisms, Diagnosis and Therapeutic Strategies. Chichester, UK: John Wiley & Sons
, pp. 207-211
-
-
Giaccone, G.1
Tagliavini, F.2
Verga, L.3
-
42
-
-
13344295093
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
55
-
-
0020450811
-
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
-
57
-
-
0015402432
-
Subacute spongiform virus encephalopathies
-
Lampert PW, Gajdusek DC, Gibbs CJ, Subacute spongiform virus encephalopathies. Scrapie, kuru and Creutzfeldt-Jakob disease: a review. Am J Pathol 1972; 68:626-652.
-
(1972)
Scrapie, Kuru and Creutzfeldt-Jakob Disease: a Review. Am J Pathol
, vol.68
, pp. 626-652
-
-
Lampert, P.W.1
Gajdusek, D.C.2
Gibbs, C.J.3
Jr4
-
58
-
-
0001238110
-
Prion diseases
-
DeArmond SJ, Prusiner SB. Prion diseases. In: Lantos P, Graham D, Lantos P, Graham DS, eds. Greenfield's neuropathology, 6th edition. London: Edward Arnold, 1997:235-280.
-
(1997)
In: Lantos P, Graham D, Lantos P, Graham DS, Eds. Greenfield's Neuropathology, 6th Edition. London: Edward Arnold
, pp. 235-280
-
-
Dearmond, S.J.1
Prusiner, S.B.2
-
60
-
-
0028102794
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
95
-
-
0029854307
-
-
Silburn P, Cervenakova L, Varghese P, et al. Fatal familial insomnia: a seventh family, Neurology 1996; 47:1326-1328.
-
(1996)
Fatal Familial Insomnia: a Seventh Family, Neurology
, vol.47
, pp. 1326-1328
-
-
Silburn, P.1
Cervenakova, L.2
Varghese, P.3
-
96
-
-
0029961971
-
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
-
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
-
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
-
100
-
-
0001935038
-
Familial thalamic degeneration with fatal insomnia: Clinicopathological and polygraphic data on a French member of Lugaresi's Italian family
-
Rancurel G, Garma L, Hauw J-J, et al. Familial thalamic degeneration with fatal insomnia: clinicopathological and polygraphic data on a French member of Lugaresi's Italian family. In: Guilleminault C, Lugaresi E, Montagna P, et al, eds. Fatal familial insomnia: inherited prion diseases, sleep, and the thalamus. New York: Raven Press, 1994:15-26.
-
(1994)
In: Guilleminault C, Lugaresi E, Montagna P, Et Al, Eds. Fatal Familial Insomnia: Inherited Prion Diseases, Sleep, and the Thalamus. New York: Raven Press
, pp. 15-26
-
-
Rancurel, G.1
Garma, L.2
Hauw, J.-J.3
-
101
-
-
0030821246
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
115
-
-
0026507882
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
124
-
-
0019801464
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
138
-
-
0001736182
-
Genotype-phenotype correlations in familial spongiform encephalopathies associated with insert mutations
-
Goldfarb LG, Cervenâkovâ L, Brown P, et al. Genotype-phenotype correlations in familial spongiform encephalopathies associated with insert mutations. In: Court L, Dodet B, Court L, Dodet BS, eds. Transmissible subacute spongiform encephalopathies: prion diseases. Paris: Eisevier, 1996:425-431.
-
(1996)
In: Court L, Dodet B, Court L, Dodet BS, Eds. Transmissible Subacute Spongiform Encephalopathies: Prion Diseases. Paris: Eisevier
, pp. 425-431
-
-
Goldfarb, L.G.1
Cervenâkovâ, L.2
Brown, P.3
-
140
-
-
0007813007
-
Inherited prion disease in Libyan Jews
-
Gabizon R, Kahana E, Hsiao K, et al. Inherited prion disease in Libyan Jews. In: Prusiner SB, Collinge J, Powell J, et al, eds. Prion diseases of humans and animals. London: Ellis Horwood, 1992:168-199.
-
(1992)
In: Prusiner SB, Collinge J, Powell J, Et Al, Eds. Prion Diseases of Humans and Animals. London: Ellis Horwood
, pp. 168-199
-
-
Gabizon, R.1
Kahana, E.2
Hsiao, K.3
-
141
-
-
0027443351
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
152
-
-
0003467727
-
Familial dementia in relation to the 144 bp insert and its implications
-
Crow TJ, Poulter M, Baker HF, et al. Familial dementia in relation to the 144 bp insert and its implications. In: Prusiner SB, Collinge J, Powell J, et al, eds. Prion diseases of humans and animals. London: Ellis Horwood, 1992:200-214.
-
(1992)
In: Prusiner SB, Collinge J, Powell J, Et Al, Eds. Prion Diseases of Humans and Animals. London: Ellis Horwood
, pp. 200-214
-
-
Crow, T.J.1
Poulter, M.2
Baker, H.F.3
-
154
-
-
0025330687
-
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
-
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
-
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
-
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
-
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
-
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
-
161
-
-
0001736182
-
Genotype-phenotype correlations in familial spongiform encephalopathies associated with insert mutations
-
Goldfarb LG, Cervenakova L, Brown P, et al. Genotype-phenotype correlations in familial spongiform encephalopathies associated with insert mutations. In: Dodet LCaB, Dodet LCaBs, eds. Transmissible subacute spongiform encephalopathies: prion diseases. Paris: Eisevier, 1996:425431.
-
(1996)
In: Dodet LCaB, Dodet LCaBs, Eds. Transmissible Subacute Spongiform Encephalopathies: Prion Diseases. Paris: Eisevier
, vol.425
, pp. 431
-
-
Goldfarb, L.G.1
Cervenakova, L.2
Brown, P.3
-
163
-
-
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, 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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
194
-
-
0343800752
-
Indiana variant of Gerstmann-Straussler-Scheinker disease
-
Ghetti B, Tagliavini F, Hsiao K, et al. Indiana variant of Gerstmann-Straussler-Scheinker disease. In: Prusiner SB, CoUinge J, Powell J, et al, eds. Prion diseases of humans and animals. London: Ellis Horwood, 1992:154-167.
-
(1992)
In: Prusiner SB, CoUinge J, Powell J, Et Al, Eds. Prion Diseases of Humans and Animals. London: Ellis Horwood
, pp. 154-167
-
-
Ghetti, B.1
Tagliavini, F.2
Hsiao, K.3
-
195
-
-
0028984802
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
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