-
1
-
-
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
-
2
-
-
0037368005
-
Perspectives on prion biology, prion disease pathogenesis, and pharmacological approaches to treatment
-
DeArmond SJ, Prusiner SB. Perspectives on prion biology, prion disease pathogenesis, and pharmacological approaches to treatment. Clinics in Laboratory Medicine 2003; 23: 1-41.
-
(2003)
Clinics in Laboratory Medicine
, vol.23
, pp. 1-41
-
-
DeArmond, S.J.1
Prusiner, S.B.2
-
3
-
-
78349247103
-
Prion amyloid structure explains templating: how proteins can be genes
-
Wickner RB, Shewmaker F, Edskes H, et al. Prion amyloid structure explains templating: how proteins can be genes. FEMS Yeast Research 2010; 10: 980-991.
-
(2010)
FEMS Yeast Research
, vol.10
, pp. 980-991
-
-
Wickner, R.B.1
Shewmaker, F.2
Edskes, H.3
-
4
-
-
5444270037
-
The persistence of long-term memory: a molecular approach to self-sustaining changes in learning-induced synaptic growth
-
Bailey CH, Kandel ER, Si K. The persistence of long-term memory: a molecular approach to self-sustaining changes in learning-induced synaptic growth. Neuron 2004; 44: 49-57.
-
(2004)
Neuron
, vol.44
, pp. 49-57
-
-
Bailey, C.H.1
Kandel, E.R.2
Si, K.3
-
5
-
-
69149088365
-
Is Parkinson's disease a prion disorder?
-
Olanow CW, Prusiner SB. Is Parkinson's disease a prion disorder? Proc Natl Acad Sci USA 2009; 106: 12571-12572.
-
(2009)
Proc Natl Acad Sci USA
, vol.106
, pp. 12571-12572
-
-
Olanow, C.W.1
Prusiner, S.B.2
-
6
-
-
77249133010
-
Prion-like mechanisms in neurodegenerative diseases
-
Frost B, Diamond MI. Prion-like mechanisms in neurodegenerative diseases. Nature Reviews Neuroscience 2010; 11: 155-159.
-
(2010)
Nature Reviews Neuroscience
, vol.11
, pp. 155-159
-
-
Frost, B.1
Diamond, M.I.2
-
7
-
-
77954385676
-
The propagation of prion-like protein inclusions in neurodegenerative diseases
-
Goedert M, Clavaguera F, Tolnay M. The propagation of prion-like protein inclusions in neurodegenerative diseases. Trends in Neurosciences 2010; 33: 317-325.
-
(2010)
Trends in Neurosciences
, vol.33
, pp. 317-325
-
-
Goedert, M.1
Clavaguera, F.2
Tolnay, M.3
-
8
-
-
77649202326
-
Protein aggregation diseases: pathogenicity and therapeutic perspectives
-
Aguzzi A, O'Connor T. Protein aggregation diseases: pathogenicity and therapeutic perspectives. Nature Reviews. Drug Discovery 2010; 9: 237-248.
-
(2010)
Nature Reviews. Drug Discovery
, vol.9
, pp. 237-248
-
-
Aguzzi, A.1
O'Connor, T.2
-
11
-
-
0032570091
-
1755 and all that: a historical primer of transmissible spongiform encephalopathy
-
Brown P, Bradley R. 1755 and all that: a historical primer of transmissible spongiform encephalopathy. BMJ 1998; 317: 1688-1692.
-
(1998)
BMJ
, vol.317
, pp. 1688-1692
-
-
Brown, P.1
Bradley, R.2
-
12
-
-
27944437441
-
Diagnosis of transmissible spongiform encephalopathies in animals: a review
-
Gavier-Widen D, Stack MJ, Baron T, Balachandran A, Simmons M. Diagnosis of transmissible spongiform encephalopathies in animals: a review. Journal of Veterinary Diagnostic Investigation 2005; 17: 509-527.
-
(2005)
Journal of Veterinary Diagnostic Investigation
, vol.17
, pp. 509-527
-
-
Gavier-Widen, D.1
Stack, M.J.2
Baron, T.3
Balachandran, A.4
Simmons, M.5
-
15
-
-
68149100194
-
Prion diseases
-
Love S, Louis DN, Ellison DW (eds). Hodder Arnold: London
-
Ironside J, Ghetti B, Head MW, Piccardo P, Will RG. Prion diseases. In Greenfiled's Neuropathology, Love S, Louis DN, Ellison DW (eds). Hodder Arnold: London, 2008; 1197-1273.
-
(2008)
Greenfiled's Neuropathology
, pp. 1197-1273
-
-
Ironside, J.1
Ghetti, B.2
Head, M.W.3
Piccardo, P.4
Will, R.G.5
-
18
-
-
0141515178
-
TSE strain variation
-
Bruce M. TSE strain variation. British Medical Bulletin 2003; 66: 99-108.
-
(2003)
British Medical Bulletin
, vol.66
, pp. 99-108
-
-
Bruce, M.1
-
19
-
-
0031942579
-
Mice with gene targeted prion protein alterations show that Prnp, Sinc and Prmi are congruent
-
Moore RC, Hope J, MacBride PA, et al. Mice with gene targeted prion protein alterations show that Prnp, Sinc and Prmi are congruent. Nature Genetics 1998; 18: 118-125.
-
(1998)
Nature Genetics
, vol.18
, pp. 118-125
-
-
Moore, R.C.1
Hope, J.2
MacBride, P.A.3
-
20
-
-
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. Journal of General Virology 1992; 73: 329-334.
-
(1992)
Journal of General Virology
, vol.73
, pp. 329-334
-
-
Bessen, R.A.1
Marsh, R.F.2
-
21
-
-
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. Journal of Virology 1994; 68: 7859-7868.
-
(1994)
Journal of Virology
, vol.68
, pp. 7859-7868
-
-
Bessen, R.A.1
Marsh, R.F.2
-
22
-
-
46749121818
-
Variably protease-sensitive prionopathy: a new sporadic disorder of the prion protein
-
Gambetti P, Dong Z, Yuan J, et al. Variably protease-sensitive prionopathy: a new sporadic disorder of the prion protein. Annals of Neurology 2008; 63: 697-708.
-
(2008)
Annals of Neurology
, vol.63
, pp. 697-708
-
-
Gambetti, P.1
Dong, Z.2
Yuan, J.3
-
23
-
-
0028876473
-
Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases)
-
Budka H, Aguzzi A, Brown P, et al. Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases). Brain Pathology 1995; 5: 457-466.
-
(1995)
Brain Pathology
, vol.5
, pp. 457-466
-
-
Budka, H.1
Aguzzi, A.2
Brown, P.3
-
24
-
-
70349947005
-
Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease
-
Zerr I, Kallenberg K, Summers DM, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 2009; 132: 2659-2668.
-
(2009)
Brain
, vol.132
, pp. 2659-2668
-
-
Zerr, I.1
Kallenberg, K.2
Summers, D.M.3
-
25
-
-
77952924225
-
Validation of diagnostic criteria for variant Creutzfeldt-Jakob disease
-
Heath CA, Cooper SA, Murray K, et al. Validation of diagnostic criteria for variant Creutzfeldt-Jakob disease. Annals of Neurology 2010; 67: 761-770.
-
(2010)
Annals of Neurology
, vol.67
, pp. 761-770
-
-
Heath, C.A.1
Cooper, S.A.2
Murray, K.3
-
26
-
-
34249937435
-
The prion protein family: diversity, rivalry, and dysfunction
-
Watts JC, Westaway D. The prion protein family: diversity, rivalry, and dysfunction. Biochemica Biophysica Acta 2007; 1772: 654-672.
-
(2007)
Biochemica Biophysica Acta
, vol.1772
, pp. 654-672
-
-
Watts, J.C.1
Westaway, D.2
-
27
-
-
57349086072
-
Association of a null allele of SPRN with variant Creutzfeldt-Jakob disease
-
Beck JA, Campbell TA, Adamson G, et al. Association of a null allele of SPRN with variant Creutzfeldt-Jakob disease. Journal of Medical Genetics 2008; 45: 813-817.
-
(2008)
Journal of Medical Genetics
, vol.45
, pp. 813-817
-
-
Beck, J.A.1
Campbell, T.A.2
Adamson, G.3
-
29
-
-
33749076227
-
Traffic of prion protein between different compartments on the neuronal surface, and the propagation of prion protein
-
Morris RJ, Parkyn CJ, Jen A. Traffic of prion protein between different compartments on the neuronal surface, and the propagation of prion protein. FEBS Letts 2006; 580: 5565-5571.
-
(2006)
FEBS Letts
, vol.580
, pp. 5565-5571
-
-
Morris, R.J.1
Parkyn, C.J.2
Jen, A.3
-
31
-
-
0012710491
-
NMR solution structure of the human prion protein
-
Zhan R, Liu A, Lurs T, et al. NMR solution structure of the human prion protein. Proc Natl Acad Sci USA 2000; 97: 145-150.
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 145-150
-
-
Zhan, R.1
Liu, A.2
Lurs, T.3
-
32
-
-
0027216665
-
Mutation and polymorphisms in the prion protein gene
-
Palmer MS, Collinge J. Mutation and polymorphisms in the prion protein gene. Human Mutation 1993; 2: 168-173.
-
(1993)
Human Mutation
, vol.2
, pp. 168-173
-
-
Palmer, M.S.1
Collinge, J.2
-
33
-
-
0033562770
-
Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease
-
Alperovitch A, Zerr I, Pocchiari M, et al. Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease. Lancet 1999; 353: 1673-1674.
-
(1999)
Lancet
, vol.353
, pp. 1673-1674
-
-
Alperovitch, A.1
Zerr, I.2
Pocchiari, M.3
-
34
-
-
33745440706
-
Kuru in the 21st century-an acquired human prion disease with very long incubation periods
-
Collinge J, Whitfield J, McKintosh E, et al. Kuru in the 21st century-an acquired human prion disease with very long incubation periods. Lancet 2006; 367: 2068-2074.
-
(2006)
Lancet
, vol.367
, pp. 2068-2074
-
-
Collinge, J.1
Whitfield, J.2
McKintosh, E.3
-
35
-
-
74549212429
-
PRNP variation in UK sporadic and variant Creutzfeldt-Jakob disease highlights genetic risk factors and a novel non-synonymous polymorphism
-
Bishop MT, Pennington C, Heath CA, Will RG, Knight RSG. PRNP variation in UK sporadic and variant Creutzfeldt-Jakob disease highlights genetic risk factors and a novel non-synonymous polymorphism. BMC Medical Genetics 2009; 10: 146.
-
(2009)
BMC Medical Genetics
, vol.10
, pp. 146
-
-
Bishop, M.T.1
Pennington, C.2
Heath, C.A.3
Will, R.G.4
Knight, R.S.G.5
-
36
-
-
77951499689
-
The genetics of prion diseases
-
Mastrianni JA. The genetics of prion diseases. Genetics in Medicine 2010; 12: 187-195.
-
(2010)
Genetics in Medicine
, vol.12
, pp. 187-195
-
-
Mastrianni, J.A.1
-
37
-
-
0032816292
-
Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects
-
Parchi P, Giese A, Capellari S, et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Annals of Neurology 1999; 46: 224-233.
-
(1999)
Annals of Neurology
, vol.46
, pp. 224-233
-
-
Parchi, P.1
Giese, A.2
Capellari, S.3
-
38
-
-
0000926966
-
Incubation period of Creutzfeldt-Jakob disease in human growth hormone recipients in France
-
Huillard d'Aignaux J, Costagliola D, Maccario J, et al. Incubation period of Creutzfeldt-Jakob disease in human growth hormone recipients in France. Neurology 1999; 53: 1197-1201.
-
(1999)
Neurology
, vol.53
, pp. 1197-1201
-
-
Huillard d'Aignaux, J.1
Costagliola, D.2
Maccario, J.3
-
39
-
-
0035863452
-
Increased susceptibility to kuru of carriers of the PRNP methionine/methionine genotype
-
Lee HS, Brown P, Cervenakova L, et al. Increased susceptibility to kuru of carriers of the PRNP methionine/methionine genotype. J Infect Disease 2001; 1834: 192-196.
-
(2001)
J Infect Disease
, vol.1834
, pp. 192-196
-
-
Lee, H.S.1
Brown, P.2
Cervenakova, L.3
-
40
-
-
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
-
42
-
-
77954102231
-
PRNP allelic series from 19years of prion protein gene sequencing at the MRC Prion Unit
-
Beck JA, Poulter M, Campbell TA, et al. PRNP allelic series from 19years of prion protein gene sequencing at the MRC Prion Unit. Human Mutation 2010; 31: E1551-E1563.
-
(2010)
Human Mutation
, vol.31
-
-
Beck, J.A.1
Poulter, M.2
Campbell, T.A.3
-
43
-
-
0029831213
-
Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
-
Collinge J, Sidle KCL, Meads J, Ironside J, Hill AF. 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
Ironside, J.4
Hill, A.F.5
-
45
-
-
12944253111
-
Genetic influence on the structural variations of the abnormal prion protein
-
Parchi P, Zou W, Wang W, et al. Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci USA 2000; 97: 10168-10172.
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 10168-10172
-
-
Parchi, P.1
Zou, W.2
Wang, W.3
-
47
-
-
2542618458
-
Prion protein heterogeneity in sporadic but not variant Creutzfeldt-Jakob diseases: UK cases 1991-2002
-
Head MW, Bunn TJR, Bishop MT, et al. Prion protein heterogeneity in sporadic but not variant Creutzfeldt-Jakob diseases: UK cases 1991-2002. Annals of Neurology 2004; 55: 851-859.
-
(2004)
Annals of Neurology
, vol.55
, pp. 851-859
-
-
Head, M.W.1
Bunn, T.J.R.2
Bishop, M.T.3
-
49
-
-
7144253121
-
Molecular pathology of fatal familial insomnia
-
Parchi P, Petersen RB, Chen SG, et al. Molecular pathology of fatal familial insomnia. Brain Pathology 1998; 8: 539-548.
-
(1998)
Brain Pathology
, vol.8
, pp. 539-548
-
-
Parchi, P.1
Petersen, R.B.2
Chen, S.G.3
-
50
-
-
0032493453
-
Differential patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Straussler-Scheinker disease
-
Parchi P, Chen SG, Brown P, et al. Differential patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Straussler-Scheinker disease. Proc Natl. Acad Sci USA 1998; 95: 8322-8327.
-
(1998)
Proc Natl. Acad Sci USA
, vol.95
, pp. 8322-8327
-
-
Parchi, P.1
Chen, S.G.2
Brown, P.3
-
51
-
-
0032975733
-
A novel phenotype in familial Creutzfeldt-Jakob disease: prion protein gene E200K mutation coupled with valine at codon 129 and type 2 protease-resistant prion protein
-
Hainfellner J, Parchi P, Kitamoto T, Jarius C, Gambetti P, Budka H. A novel phenotype in familial Creutzfeldt-Jakob disease: prion protein gene E200K mutation coupled with valine at codon 129 and type 2 protease-resistant prion protein. Annals of Neurology 1999; 45: 812-816.
-
(1999)
Annals of Neurology
, vol.45
, pp. 812-816
-
-
Hainfellner, J.1
Parchi, P.2
Kitamoto, T.3
Jarius, C.4
Gambetti, P.5
Budka, H.6
-
52
-
-
0141514771
-
Sporadic and familial CJD: classification and characterisation
-
Gambetti P, Kong Q, Zou W, Parchi P, Chen SG. Sporadic and familial CJD: classification and characterisation. British Medical Bulletin 2003; 66: 213-239.
-
(2003)
British Medical Bulletin
, vol.66
, pp. 213-239
-
-
Gambetti, P.1
Kong, Q.2
Zou, W.3
Parchi, P.4
Chen, S.G.5
-
53
-
-
33244468109
-
Distinct glycoform ratios of protease resistant prion protein associated with PRNP point mutations
-
Hill AF, Joiner S, Beck JA, et al. Distinct glycoform ratios of protease resistant prion protein associated with PRNP point mutations. Brain 2006; 129: 676-685.
-
(2006)
Brain
, vol.129
, pp. 676-685
-
-
Hill, A.F.1
Joiner, S.2
Beck, J.A.3
-
54
-
-
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
-
55
-
-
79955759823
-
Diagnosing variant Creutzfeldt-Jakob disease: a retrospective analysis of the first 150 cases in the UK
-
Heath CA, Cooper SA, Murray K, et al. Diagnosing variant Creutzfeldt-Jakob disease: a retrospective analysis of the first 150 cases in the UK. Journal of Neurology, Neurosurgery, and Psychiatry 2011; 82: 646-651.
-
(2011)
Journal of Neurology, Neurosurgery, and Psychiatry
, vol.82
, pp. 646-651
-
-
Heath, C.A.1
Cooper, S.A.2
Murray, K.3
-
56
-
-
0033930868
-
Laboratory diagnosis of variant Creutzfeldt-Jakob disease
-
Ironside JW, Head MW, Bell JE, McCardle L, Will RG. Laboratory diagnosis of variant Creutzfeldt-Jakob disease. Histopathol 2000; 37: 1-9.
-
(2000)
Histopathol
, vol.37
, pp. 1-9
-
-
Ironside, J.W.1
Head, M.W.2
Bell, J.E.3
McCardle, L.4
Will, R.G.5
-
57
-
-
0032578283
-
Prion immunoreactivity in appendix before clinical onset of variant Creutzfeldt-Jakob disease
-
Hilton DA, Fathers E, Edwards P, Ironside JW, Zajicek J. Prion immunoreactivity in appendix before clinical onset of variant Creutzfeldt-Jakob disease. Lancet 1998; 352: 703-704.
-
(1998)
Lancet
, vol.352
, pp. 703-704
-
-
Hilton, D.A.1
Fathers, E.2
Edwards, P.3
Ironside, J.W.4
Zajicek, J.5
-
58
-
-
0033573778
-
Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples
-
Hill AF, Butterworth RJ, Joiner S, et al. Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet 1999; 353: 183-189.
-
(1999)
Lancet
, vol.353
, pp. 183-189
-
-
Hill, A.F.1
Butterworth, R.J.2
Joiner, S.3
-
59
-
-
0035928432
-
Tissue distribution of protease resistant prion in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay
-
Wadsworth JDF, Joiner S, Hill AF, et al. Tissue distribution of protease resistant prion in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet 2001; 358: 171-180.
-
(2001)
Lancet
, vol.358
, pp. 171-180
-
-
Wadsworth, J.D.F.1
Joiner, S.2
Hill, A.F.3
-
60
-
-
1142267004
-
Peripheral tissue involvement in sporadic, iatrogenic, and variant Creutzfeldt-Jakob disease: an immunocytochemical, quantitative and biochemical study
-
Head MW, Ritchie D, Smith N, et al. Peripheral tissue involvement in sporadic, iatrogenic, and variant Creutzfeldt-Jakob disease: an immunocytochemical, quantitative and biochemical study. American Journal of Pathology 2004; 164: 143-153.
-
(2004)
American Journal of Pathology
, vol.164
, pp. 143-153
-
-
Head, M.W.1
Ritchie, D.2
Smith, N.3
-
61
-
-
72249109696
-
Variant CJD in an individual heterozygous for PRNP codon 129
-
Kaski D, Mead S, Hyare H, et al. Variant CJD in an individual heterozygous for PRNP codon 129. Lancet 2009; 374: 2128.
-
(2009)
Lancet
, vol.374
, pp. 2128
-
-
Kaski, D.1
Mead, S.2
Hyare, H.3
-
62
-
-
33748369869
-
Creutzfeldt-Jakob disease and blood transfusion: results of the UK transfusion medicine epidemiology review study
-
Hewitt PE, Llewelyn CA, Mackenzie J, Will RG. Creutzfeldt-Jakob disease and blood transfusion: results of the UK transfusion medicine epidemiology review study. Vox Sanguinus 2006; 91: 221-230.
-
(2006)
Vox Sanguinus
, vol.91
, pp. 221-230
-
-
Hewitt, P.E.1
Llewelyn, C.A.2
Mackenzie, J.3
Will, R.G.4
-
63
-
-
84863544528
-
-
-
-
-
64
-
-
33845227845
-
Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report
-
Wroe SJ, Pal S, Siddique D, et al. Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report. Lancet 2006; 368: 2061-2067.
-
(2006)
Lancet
, vol.368
, pp. 2061-2067
-
-
Wroe, S.J.1
Pal, S.2
Siddique, D.3
-
65
-
-
67649995088
-
Pathological investigation of the first blood donor and recipient pair linked by transfusion-associated variant Creutzfeldt-Jakob disease
-
Head MW, Yull HM, Ritchie DL, Bishop MT, Ironside JW. Pathological investigation of the first blood donor and recipient pair linked by transfusion-associated variant Creutzfeldt-Jakob disease. Neuropathol Applied Neurobiol 2009; 35: 433-436.
-
(2009)
Neuropathol Applied Neurobiol
, vol.35
, pp. 433-436
-
-
Head, M.W.1
Yull, H.M.2
Ritchie, D.L.3
Bishop, M.T.4
Ironside, J.W.5
-
66
-
-
4043157677
-
Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient
-
Peden AH, Head MW, Ritchie DR, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004; 364: 527-529.
-
(2004)
Lancet
, vol.364
, pp. 527-529
-
-
Peden, A.H.1
Head, M.W.2
Ritchie, D.R.3
Bell, J.E.4
Ironside, J.W.5
-
67
-
-
77749322732
-
Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia
-
Peden A, McCardle L, Head MW, et al. Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia. Haemophilia 2010; 16: 296-304.
-
(2010)
Haemophilia
, vol.16
, pp. 296-304
-
-
Peden, A.1
McCardle, L.2
Head, M.W.3
-
68
-
-
33646898809
-
Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study
-
Ironside JW, Bishop MT, Connolly K, et al. Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. BMJ 2006; 332: 1186-1188.
-
(2006)
BMJ
, vol.332
, pp. 1186-1188
-
-
Ironside, J.W.1
Bishop, M.T.2
Connolly, K.3
-
69
-
-
65249149613
-
Variant Creutzfeldt-Jakob disease in France and the United Kingdom: evidence for the same agent strain
-
Brandel JP, Heath CA, Head MW, et al. Variant Creutzfeldt-Jakob disease in France and the United Kingdom: evidence for the same agent strain. Annals of Neurology 2009; 65: 249-256.
-
(2009)
Annals of Neurology
, vol.65
, pp. 249-256
-
-
Brandel, J.P.1
Heath, C.A.2
Head, M.W.3
-
70
-
-
77649280662
-
Multiorgan detection and characterisation of protease-resistant prion protein in a case of variant CJD examined in the United States
-
Notari S, Moleres F, Hunter SB, et al. Multiorgan detection and characterisation of protease-resistant prion protein in a case of variant CJD examined in the United States. PLoS One 2010; 5: e8765.
-
(2010)
PLoS One
, vol.5
-
-
Notari, S.1
Moleres, F.2
Hunter, S.B.3
-
71
-
-
0034909904
-
Sporadic Creutzfeldt-Jakob disease in a young Dutch valine homozygote: atypical molecular phenotype
-
Head MW, Tissingh G, Uitdenhaag BMJ, et al. Sporadic Creutzfeldt-Jakob disease in a young Dutch valine homozygote: atypical molecular phenotype. Annals of Neurology 2001; 50: 258-261.
-
(2001)
Annals of Neurology
, vol.50
, pp. 258-261
-
-
Head, M.W.1
Tissingh, G.2
Uitdenhaag, B.M.J.3
-
72
-
-
50949100224
-
res glycosylation in Creutzfeldt-Jakob disease-implications for the dissemination and diagnosis of human prion strains
-
res glycosylation in Creutzfeldt-Jakob disease-implications for the dissemination and diagnosis of human prion strains. PLoS One 2008; 3: e2786.
-
(2008)
PLoS One
, vol.3
-
-
Levavasseur, E.1
Laffont-Proust, I.2
Morain, E.3
-
74
-
-
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
-
75
-
-
0033592877
-
Compelling transgenic evidence for transmission of bovine spongiform encephalopathy prions to humans
-
Scott MR, Will R, Ironside J, et al. Compelling transgenic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci USA 1999; 96: 15137-15142.
-
(1999)
Proc Natl Acad Sci USA
, vol.96
, pp. 15137-15142
-
-
Scott, M.R.1
Will, R.2
Ironside, J.3
-
76
-
-
70849090446
-
Transmission of variant Creutzfeldt-Jakob disease from brain and lymphoreticular tissue show uniform and conserved bovine spongiform encephalopathy-related phenotypic properties on primary and secondary passage in wild-type mice
-
Ritchie DL, Boyle A, McConnell I, Head MW, Ironside JW, Bruce ME. Transmission of variant Creutzfeldt-Jakob disease from brain and lymphoreticular tissue show uniform and conserved bovine spongiform encephalopathy-related phenotypic properties on primary and secondary passage in wild-type mice. Journal of General Virology 2009; 90: 3075-3082.
-
(2009)
Journal of General Virology
, vol.90
, pp. 3075-3082
-
-
Ritchie, D.L.1
Boyle, A.2
McConnell, I.3
Head, M.W.4
Ironside, J.W.5
Bruce, M.E.6
-
77
-
-
33646059507
-
Predicting susceptibility and incubation time of human-to-human transmission of vCJD
-
Bishop MT, Hart P, Aitchison L, et al. Predicting susceptibility and incubation time of human-to-human transmission of vCJD. Lancet Neurology 2006; 5: 393-398.
-
(2006)
Lancet Neurology
, vol.5
, pp. 393-398
-
-
Bishop, M.T.1
Hart, P.2
Aitchison, L.3
-
78
-
-
51449104990
-
No major change in vCJD agent strain after secondary transmission via blood transfusion
-
Bishop MT, Ritchie DR, Will RG, et al. No major change in vCJD agent strain after secondary transmission via blood transfusion. PLoS One 2008; 3: e2878.
-
(2008)
PLoS One
, vol.3
-
-
Bishop, M.T.1
Ritchie, D.R.2
Will, R.G.3
-
80
-
-
57149144250
-
Sc type and PRNP genotype in an in-vitro conversion assay
-
Sc type and PRNP genotype in an in-vitro conversion assay. NeuroReport 2008; 19: 1783-1786.
-
(2008)
NeuroReport
, vol.19
, pp. 1783-1786
-
-
Jones, M.1
Peden, A.H.2
Wight, D.3
-
81
-
-
58849146750
-
Human platelets as a substrate source for the in vitro amplification of abnormal prion protein (PrP) associated with variant Creutzfeldt-Jakob disease
-
Jones M, Peden AH, Yull H, et al. Human platelets as a substrate source for the in vitro amplification of abnormal prion protein (PrP) associated with variant Creutzfeldt-Jakob disease. Transfusion 2009; 49: 376-384.
-
(2009)
Transfusion
, vol.49
, pp. 376-384
-
-
Jones, M.1
Peden, A.H.2
Yull, H.3
-
83
-
-
0032976574
-
A subtype of sporadic prion disease mimicking fatal familial insomnia
-
Parchi P, Capellari S, Chin S, et al. A subtype of sporadic prion disease mimicking fatal familial insomnia. Neurology 1999; 52: 1757-1763.
-
(1999)
Neurology
, vol.52
, pp. 1757-1763
-
-
Parchi, P.1
Capellari, S.2
Chin, S.3
-
84
-
-
2942694110
-
and the WHO Working Group in international reference materials for the diagnosis and study of transmissible spongiform encephalopathies
-
Minor P, Newham J, Jones N, et al. and the WHO Working Group in international reference materials for the diagnosis and study of transmissible spongiform encephalopathies. Journal of General Virology 2004; 85: 1777-1784.
-
(2004)
Journal of General Virology
, vol.85
, pp. 1777-1784
-
-
Minor, P.1
Newham, J.2
Jones, N.3
-
88
-
-
70349937836
-
Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics
-
Cali I, Castellani R, Alshekhlee A, et al. Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics. Brain 2009: 132: 2643-2658.
-
(2009)
Brain
, vol.132
, pp. 2643-2658
-
-
Cali, I.1
Castellani, R.2
Alshekhlee, A.3
-
92
-
-
70349952066
-
Sporadic Creutzfeldt-Jakob disease: discrete subtypes or a spectrum of disease?
-
Head MW, Ironside JW. Sporadic Creutzfeldt-Jakob disease: discrete subtypes or a spectrum of disease? Brain 2009; 132: 2629-2629.
-
(2009)
Brain
, vol.132
, pp. 2629-2629
-
-
Head, M.W.1
Ironside, J.W.2
-
93
-
-
77955344991
-
Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties
-
Bishop MT, Will RG, Manson JC. Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc Natl Acad Sci USA 2010; 107: 12005-12010.
-
(2010)
Proc Natl Acad Sci USA
, vol.107
, pp. 12005-12010
-
-
Bishop, M.T.1
Will, R.G.2
Manson, J.C.3
-
94
-
-
70449709025
-
A case of protease sensitive prionopathy in a patient in the UK
-
Head MW, Knight R, Zeidler M, Yull H, Barlow A, Ironside JW. A case of protease sensitive prionopathy in a patient in the UK. Neuropathol Applied Neurobiol 2009; 35: 628-632.
-
(2009)
Neuropathol Applied Neurobiol
, vol.35
, pp. 628-632
-
-
Head, M.W.1
Knight, R.2
Zeidler, M.3
Yull, H.4
Barlow, A.5
Ironside, J.W.6
-
95
-
-
77955295345
-
The first case of protease-sensitive prionopathy (PSPr) in the Netherlands: a patient with an unusual GSS-like clinical presentation
-
Jansen C, Head MW, van Gool WA, et al. The first case of protease-sensitive prionopathy (PSPr) in the Netherlands: a patient with an unusual GSS-like clinical presentation. Journal of Neurology, Neurosurgery, and Psychiatry 2010; 81: 1052-1055.
-
(2010)
Journal of Neurology, Neurosurgery, and Psychiatry
, vol.81
, pp. 1052-1055
-
-
Jansen, C.1
Head, M.W.2
van Gool, W.A.3
-
96
-
-
77955302607
-
Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein
-
Zou WQ, Puoti G, Xiao X, et al. Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Annals of Neurology 2010; 68: 162-172.
-
(2010)
Annals of Neurology
, vol.68
, pp. 162-172
-
-
Zou, W.Q.1
Puoti, G.2
Xiao, X.3
-
97
-
-
36849086384
-
A novel phenotype of sporadic Creutzfeldt-Jakob disease
-
Giaccone G, Di Fede G, Mangieri M, et al. A novel phenotype of sporadic Creutzfeldt-Jakob disease. Journal of Neurology, Neurosurgery, and Psychiatry 2007; 78: 1379-1382.
-
(2007)
Journal of Neurology, Neurosurgery, and Psychiatry
, vol.78
, pp. 1379-1382
-
-
Giaccone, G.1
Di Fede, G.2
Mangieri, M.3
-
98
-
-
78651068014
-
Variably protease-sensitive prionopathy in a PRNP heterozygous UK patient with co-existing tau, α-synuclein and Aβ pathology
-
Head MW, Lowrie S, Chohan G, Knight R, Scoones DJ, Ironside JW. Variably protease-sensitive prionopathy in a PRNP heterozygous UK patient with co-existing tau, α-synuclein and Aβ pathology. Acta Neuropathologica 2010; 120: 821-823.
-
(2010)
Acta Neuropathologica
, vol.120
, pp. 821-823
-
-
Head, M.W.1
Lowrie, S.2
Chohan, G.3
Knight, R.4
Scoones, D.J.5
Ironside, J.W.6
-
99
-
-
79952113179
-
A novel form of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: case report
-
Rodriguez-Martinez AB, Garrido JM, Zarranz JJ, et al. A novel form of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: case report. BMC Neurology 2010; 10: 99.
-
(2010)
BMC Neurology
, vol.10
, pp. 99
-
-
Rodriguez-Martinez, A.B.1
Garrido, J.M.2
Zarranz, J.J.3
-
100
-
-
85046172265
-
Creutzfeldt-Jakob disease with florid plaques after cadaveric dural graft in a Japanese woman
-
Takashima S, Tateishi J, Taguchi Y, Inoue H. Creutzfeldt-Jakob disease with florid plaques after cadaveric dural graft in a Japanese woman. Lancet 1977; 350: 865-866.
-
(1977)
Lancet
, vol.350
, pp. 865-866
-
-
Takashima, S.1
Tateishi, J.2
Taguchi, Y.3
Inoue, H.4
-
101
-
-
0028211267
-
Iatrogenic Creutzfeldt-Jakob disease in three growth hormone recipients: a neuropathological study
-
Billette de Villemeur T, Gelot A, Deslys JP, et al. Iatrogenic Creutzfeldt-Jakob disease in three growth hormone recipients: a neuropathological study. Neuropathol Applied Neurobiol 1994; 20: 111-117.
-
(1994)
Neuropathol Applied Neurobiol
, vol.20
, pp. 111-117
-
-
Billette de Villemeur, T.1
Gelot, A.2
Deslys, J.P.3
-
102
-
-
0032565362
-
Genotype at codon 129 and susceptibility to Creutzfeldt-Jakob disease
-
Deslys JP, Jaegly A, Huillard d'Aignaux J, Mouthon F, Billette de Villemeur T, Dormond D. Genotype at codon 129 and susceptibility to Creutzfeldt-Jakob disease. Lancet 1998; 351: 1251.
-
(1998)
Lancet
, vol.351
, pp. 1251
-
-
Deslys, J.P.1
Jaegly, A.2
Huillard d'Aignaux, J.3
Mouthon, F.4
Billette de Villemeur, T.5
Dormond, D.6
-
103
-
-
0141841804
-
Association of an 11-12kDa protease-resistant prion protein fragment with subtypes of dura mater graft-associated Creutzfeldt-Jakob disease and other prion diseases
-
Satoh K, Muramato T, Tanaka T, et al. Association of an 11-12kDa protease-resistant prion protein fragment with subtypes of dura mater graft-associated Creutzfeldt-Jakob disease and other prion diseases. Journal of General Virology 2003; 84: 2885-2893.
-
(2003)
Journal of General Virology
, vol.84
, pp. 2885-2893
-
-
Satoh, K.1
Muramato, T.2
Tanaka, T.3
-
104
-
-
33745311131
-
Dura mater-associated Creutzfeldt-Jakob disease: experience from surveillance in the UK
-
Heath CA, Barker RA, Esmonde TFG, et al. Dura mater-associated Creutzfeldt-Jakob disease: experience from surveillance in the UK. Journal of Neurology, Neurosurgery, and Psychiatry 2006; 77: 880-882.
-
(2006)
Journal of Neurology, Neurosurgery, and Psychiatry
, vol.77
, pp. 880-882
-
-
Heath, C.A.1
Barker, R.A.2
Esmonde, T.F.G.3
-
105
-
-
0038304770
-
Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UK
-
Brandel JP, Preece M, Brown P, et al. Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UK. Lancet 2003; 362: 129-130.
-
(2003)
Lancet
, vol.362
, pp. 129-130
-
-
Brandel, J.P.1
Preece, M.2
Brown, P.3
-
106
-
-
0034711176
-
Iatrogenic Creutzfeldt-Jakob disease at the millennium
-
Brown P, Preece M, Brandel JP, et al. Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology 2000; 55: 1075-1081.
-
(2000)
Neurology
, vol.55
, pp. 1075-1081
-
-
Brown, P.1
Preece, M.2
Brandel, J.P.3
-
107
-
-
33747040538
-
Iatrogenic Creutzfeldt-Jakob disease: the waning of an era
-
Brown P, Brandel JP, Preece M, Sato T. Iatrogenic Creutzfeldt-Jakob disease: the waning of an era. Neurology 2006; 67: 389-393.
-
(2006)
Neurology
, vol.67
, pp. 389-393
-
-
Brown, P.1
Brandel, J.P.2
Preece, M.3
Sato, T.4
-
108
-
-
0035957401
-
Adaptation of bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt-Jakob disease: implications for human health
-
Lasmezas CI, Fournier JG, Nouvel V, et al. Adaptation of bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt-Jakob disease: implications for human health. Proc Natl Acad Sci USA 2001; 98: 4142-4147.
-
(2001)
Proc Natl Acad Sci USA
, vol.98
, pp. 4142-4147
-
-
Lasmezas, C.I.1
Fournier, J.G.2
Nouvel, V.3
-
109
-
-
41649086573
-
Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice
-
Wadsworth JDF, Joiner S, Lineham JM, et al. Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. Proc Natl Acad Sci USA 2008; 105: 3885-3890.
-
(2008)
Proc Natl Acad Sci USA
, vol.105
, pp. 3885-3890
-
-
Wadsworth, J.D.F.1
Joiner, S.2
Lineham, J.M.3
-
110
-
-
0036459944
-
Mutations of the prion protein gene: phenotypic spectrum
-
Kovacs GG, Trabattonni G, Hainfellner JA, Ironside JW, Knight RSG, Budka H. Mutations of the prion protein gene: phenotypic spectrum. Journal of Neurology 2002; 249: 1567-1582.
-
(2002)
Journal of Neurology
, vol.249
, pp. 1567-1582
-
-
Kovacs, G.G.1
Trabattonni, G.2
Hainfellner, J.A.3
Ironside, J.W.4
Knight, R.S.G.5
Budka, H.6
-
111
-
-
78651246462
-
Genetic Creutzfeldt-Jakob disease and fatal familial insomnia: insights into phenotypic variability and disease pathogenesis
-
Capellari S, Strammiello R, Saverioni D, Kretzschmar H, Parchi P. Genetic Creutzfeldt-Jakob disease and fatal familial insomnia: insights into phenotypic variability and disease pathogenesis. Acta Neuropathologica 2011; 121: 21-37.
-
(2011)
Acta Neuropathologica
, vol.121
, pp. 21-37
-
-
Capellari, S.1
Strammiello, R.2
Saverioni, D.3
Kretzschmar, H.4
Parchi, P.5
-
112
-
-
0034234437
-
Intracerebral distribution of the abnormal isoform of the prion protein in sporadic Creutzfeldt-Jakob disease and fatal insomnia
-
Parchi P, Capellari S, Gambetti P. Intracerebral distribution of the abnormal isoform of the prion protein in sporadic Creutzfeldt-Jakob disease and fatal insomnia. Micro Res Tech 2000; 50: 16-25.
-
(2000)
Micro Res Tech
, vol.50
, pp. 16-25
-
-
Parchi, P.1
Capellari, S.2
Gambetti, P.3
-
113
-
-
10344259077
-
Proteinase-K-resistant prion protein isoforms in Gerstmann-Straussler-Scheinker disease (Indiana kindred)
-
Piccardo P, Seiler C, Dlouhy SR, et al. Proteinase-K-resistant prion protein isoforms in Gerstmann-Straussler-Scheinker disease (Indiana kindred). Journal of Neuropathology and Experimental Neurology 1996; 55: 1157-1163.
-
(1996)
Journal of Neuropathology and Experimental Neurology
, vol.55
, pp. 1157-1163
-
-
Piccardo, P.1
Seiler, C.2
Dlouhy, S.R.3
-
114
-
-
0031754291
-
Phenotypic variability of Gerstmann-Straussler-Scheinker disease is associated with prion protein heterogeneity
-
Piccardo P, Dlouhy SR, Lievens PMJ, et al. Phenotypic variability of Gerstmann-Straussler-Scheinker disease is associated with prion protein heterogeneity. Journal of Neuropathology and Experimental Neurology 1998; 57: 979-988.
-
(1998)
Journal of Neuropathology and Experimental Neurology
, vol.57
, pp. 979-988
-
-
Piccardo, P.1
Dlouhy, S.R.2
Lievens, P.M.J.3
-
115
-
-
0034974318
-
Prion proteins with different conformations accumulate in Gerstmann-Straussler-Scheinker disease caused by A117V and F198S mutations
-
Piccardo P, Liepnieks JJ, Willaim A, et al. Prion proteins with different conformations accumulate in Gerstmann-Straussler-Scheinker disease caused by A117V and F198S mutations. American Journal of Pathology 2001; 158: 2201-2207.
-
(2001)
American Journal of Pathology
, vol.158
, pp. 2201-2207
-
-
Piccardo, P.1
Liepnieks, J.J.2
Willaim, A.3
-
116
-
-
9644299705
-
sc heterogeneity in inherited prion diseases with the D178N mutation
-
sc heterogeneity in inherited prion diseases with the D178N mutation. Annals of Neurology 2004; 57: 909-910.
-
(2004)
Annals of Neurology
, vol.57
, pp. 909-910
-
-
Haik, S.1
Peoc'h, K.2
Brandel, J.P.3
-
117
-
-
78651257339
-
Genetic Creutzfeldt-Jakob disease associated with the E200K mutation: characterisation of a complex proteinopathy
-
Kovacs GG, Seguin J, Quadrio I, et al. Genetic Creutzfeldt-Jakob disease associated with the E200K mutation: characterisation of a complex proteinopathy. Acta Neuropathologica 2011; 121: 39-57.
-
(2011)
Acta Neuropathologica
, vol.121
, pp. 39-57
-
-
Kovacs, G.G.1
Seguin, J.2
Quadrio, I.3
-
118
-
-
0030768832
-
Allelic origin of the abnormal prion protein isoform in familial prion diseases
-
Chen SG, Parchi P, Brown P, et al. Allelic origin of the abnormal prion protein isoform in familial prion diseases. Nature Med 1997: 3: 1009-1015.
-
(1997)
Nature Med
, vol.3
, pp. 1009-1015
-
-
Chen, S.G.1
Parchi, P.2
Brown, P.3
-
119
-
-
33745094097
-
Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein
-
Wadsworth JDF, Joiner S, Lineham JM, et al. Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein. Brain 2006; 129: 1557-1569.
-
(2006)
Brain
, vol.129
, pp. 1557-1569
-
-
Wadsworth, J.D.F.1
Joiner, S.2
Lineham, J.M.3
-
120
-
-
12644272790
-
Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity
-
Telling GC, Parchi P, DeArmond SJ, et al. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science 1996; 274: 2079-2082.
-
(1996)
Science
, vol.274
, pp. 2079-2082
-
-
Telling, G.C.1
Parchi, P.2
DeArmond, S.J.3
-
121
-
-
0033609313
-
Prion protein conformation in a patient with sporadic fatal insomnia
-
Mastrianni J, Nixon R, Layzer R, et al. Prion protein conformation in a patient with sporadic fatal insomnia. New Eng J Med 1999; 340: 1630-1638.
-
(1999)
New Eng J Med
, vol.340
, pp. 1630-1638
-
-
Mastrianni, J.1
Nixon, R.2
Layzer, R.3
-
123
-
-
34248396416
-
Accumulation of prion protein in the brain that is not associated with transmissible disease
-
Picardo P, Manson JC, King D, Ghetti B, Barron RM. Accumulation of prion protein in the brain that is not associated with transmissible disease. Proc Natl Acad Sci USA 2007; 104: 4712-4717.
-
(2007)
Proc Natl Acad Sci USA
, vol.104
, pp. 4712-4717
-
-
Picardo, P.1
Manson, J.C.2
King, D.3
Ghetti, B.4
Barron, R.M.5
-
124
-
-
20344394154
-
Anchorless prion protein results in infectious amyloid disease without clinical scrapie
-
Chesebro B, Trifilo M, Race R, et al. Anchorless prion protein results in infectious amyloid disease without clinical scrapie. Science 2005; 308: 1435-1439.
-
(2005)
Science
, vol.308
, pp. 1435-1439
-
-
Chesebro, B.1
Trifilo, M.2
Race, R.3
-
125
-
-
77950379326
-
Fatal transmissible amyloid encephalopathy: a new type of prion disease associated with lack of prion protein anchoring
-
Chesebro B, Race B, Meade-White K, et al. Fatal transmissible amyloid encephalopathy: a new type of prion disease associated with lack of prion protein anchoring. PLoS Pathogens 2010; 6: e1000800.
-
(2010)
PLoS Pathogens
, vol.6
-
-
Chesebro, B.1
Race, B.2
Meade-White, K.3
-
126
-
-
13344295093
-
Vascular variant of prion protein cerebral amyloidosis with τ-positive 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 τ-positive 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
-
127
-
-
84861708037
-
Clinicopathological observations associated with a novel truncated mutation of PRNP
-
Holton JL, Mead S, Gandhi S, et al. Clinicopathological observations associated with a novel truncated mutation of PRNP. Neuropathol Applied Neurobiol 2010; 36(Suppl. 1): 2.
-
(2010)
Neuropathol Applied Neurobiol
, vol.36
, Issue.SUPPL. 1
, pp. 2
-
-
Holton, J.L.1
Mead, S.2
Gandhi, S.3
-
128
-
-
77449089995
-
Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNP
-
Jansen C, Parchi P, Capellari S, et al. Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNP. Acta Neuropathologica 2010; 119: 189-197.
-
(2010)
Acta Neuropathologica
, vol.119
, pp. 189-197
-
-
Jansen, C.1
Parchi, P.2
Capellari, S.3
-
129
-
-
79955397626
-
Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype
-
Jayadev S, Nochlin D, Poorkaj P, et al. Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype. Annals of Neurology 2011; 69: 712-720.
-
(2011)
Annals of Neurology
, vol.69
, pp. 712-720
-
-
Jayadev, S.1
Nochlin, D.2
Poorkaj, P.3
-
130
-
-
0035813151
-
Novel differences between two human prion strains revealed by two-dimensional gel electrophoresis
-
Pan T, Colucci M, Wong BS, et al. Novel differences between two human prion strains revealed by two-dimensional gel electrophoresis. Journal of Biological Chemistry 2001; 276: 37284-37288.
-
(2001)
Journal of Biological Chemistry
, vol.276
, pp. 37284-37288
-
-
Pan, T.1
Colucci, M.2
Wong, B.S.3
-
132
-
-
34247185404
-
Disease-associated prion protein oligomers inhibit the 26S proteasome
-
Kristiansen M, Deriziotis P, Dimcheff DE, et al. Disease-associated prion protein oligomers inhibit the 26S proteasome. Molecular Cell 2007; 26: 175-188.
-
(2007)
Molecular Cell
, vol.26
, pp. 175-188
-
-
Kristiansen, M.1
Deriziotis, P.2
Dimcheff, D.E.3
-
133
-
-
68849128369
-
Development of oligomeric prion protein aggregates in a mouse model of prion disease
-
Sasaki K, Minaki H, Iwaki T. Development of oligomeric prion protein aggregates in a mouse model of prion disease. The Journal of Pathology 2009; 219: 123-130.
-
(2009)
The Journal of Pathology
, vol.219
, pp. 123-130
-
-
Sasaki, K.1
Minaki, H.2
Iwaki, T.3
-
135
-
-
33749254251
-
Classification of sporadic Creutzfeldt-Jakob disease revisited
-
Cali I, Castellani R, Yuan J, et al. Classification of sporadic Creutzfeldt-Jakob disease revisited. Brain 2006; 129: 2266-2277.
-
(2006)
Brain
, vol.129
, pp. 2266-2277
-
-
Cali, I.1
Castellani, R.2
Yuan, J.3
-
136
-
-
33845925066
-
Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brains
-
Yuan J, Xiao X, McGeehan J, et al. Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brains. Journal of Biological Chemistry 2006; 281: 34848-34858.
-
(2006)
Journal of Biological Chemistry
, vol.281
, pp. 34848-34858
-
-
Yuan, J.1
Xiao, X.2
McGeehan, J.3
-
137
-
-
79954611509
-
Correlation of polydispered prion protein and characteristic pathology in the thalamus in variant Creutzfeldt-Jakob disease: implication of small oligomeric species
-
Choi YP, Groener A, Ironside JW, Head MW. Correlation of polydispered prion protein and characteristic pathology in the thalamus in variant Creutzfeldt-Jakob disease: implication of small oligomeric species. Brain Pathology 2011; 21: 298-307.
-
(2011)
Brain Pathology
, vol.21
, pp. 298-307
-
-
Choi, Y.P.1
Groener, A.2
Ironside, J.W.3
Head, M.W.4
-
139
-
-
79951802675
-
Comparison of the level, distribution and form of disease-associated prion protein in variant and sporadic Creutzfeldt-Jakob disease brain using conformation-dependent immunoassay and Western blot
-
Choi YP, Groener A, Ironside JW, Head MW. Comparison of the level, distribution and form of disease-associated prion protein in variant and sporadic Creutzfeldt-Jakob disease brain using conformation-dependent immunoassay and Western blot. Journal of General Virology 2011; 92: 727-732.
-
(2011)
Journal of General Virology
, vol.92
, pp. 727-732
-
-
Choi, Y.P.1
Groener, A.2
Ironside, J.W.3
Head, M.W.4
-
140
-
-
0031720905
-
Sc molecules with different conformations
-
Sc molecules with different conformations. Nature Med 1998; 4: 1157-1165.
-
(1998)
Nature Med
, vol.4
, pp. 1157-1165
-
-
Safar, J.1
Wille, H.2
Itra, V.3
-
141
-
-
34447639732
-
Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes
-
Legname G, Nguyen HOB, Peretz D, Cohen FE, DeArmond SJ, Prusiner SB. Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes. Proc Natl Acad Sci USA 2006; 103: 19105-19110.
-
(2006)
Proc Natl Acad Sci USA
, vol.103
, pp. 19105-19110
-
-
Legname, G.1
Nguyen, H.O.B.2
Peretz, D.3
Cohen, F.E.4
DeArmond, S.J.5
Prusiner, S.B.6
-
142
-
-
73949160065
-
Design and construction of diverse mammalian prion strains
-
Colby DW, Giles K, Legname G, et al. Design and construction of diverse mammalian prion strains. Proc Natl Acad Sci USA 2009; 106: 20417-20422.
-
(2009)
Proc Natl Acad Sci USA
, vol.106
, pp. 20417-20422
-
-
Colby, D.W.1
Giles, K.2
Legname, G.3
-
143
-
-
78049519681
-
Distinct stability states of disease-associated human prion protein identified by conformation-dependent immunoassay
-
Choi YP, Peden AH, Groener A, Ironside JW, Head MW. Distinct stability states of disease-associated human prion protein identified by conformation-dependent immunoassay. Journal of Virology 2010; 84: 12030-12038.
-
(2010)
Journal of Virology
, vol.84
, pp. 12030-12038
-
-
Choi, Y.P.1
Peden, A.H.2
Groener, A.3
Ironside, J.W.4
Head, M.W.5
-
145
-
-
33749632465
-
Protein misfolding cyclic amplification for diagnosis and prion propagation studies
-
Castilla J, Saa P, Morales R, Abid K, Maudrell K, Soto C. Protein misfolding cyclic amplification for diagnosis and prion propagation studies. Methods in Enzymology 2006; 412: 3-21.
-
(2006)
Methods in Enzymology
, vol.412
, pp. 3-21
-
-
Castilla, J.1
Saa, P.2
Morales, R.3
Abid, K.4
Maudrell, K.5
Soto, C.6
|