Shattuck lecture - Neurodegenerative diseases and prions

New England Journal of Medicine

Volumn 344, Issue 20, 2001, Pages 1516-1526

  Prusiner, Stanley B ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

DENDRIMER; LEVODOPA; PRION PROTEIN;

ALZHEIMER DISEASE; AMYOTROPHIC LATERAL SCLEROSIS; ARTICLE; CLINICAL FEATURE; DEGENERATIVE DISEASE; DIAGNOSTIC TEST; FRONTOTEMPORAL DEMENTIA; HUMAN; HUMAN TISSUE; HUNTINGTON CHOREA; NEUROPATHOLOGY; PARKINSON DISEASE; PICK PRESENILE DEMENTIA; PREVALENCE; PRION DISEASE; PRIORITY JOURNAL; SPINOCEREBELLAR DEGENERATION; SUBSTITUTION THERAPY;

AGE FACTORS; DIAGNOSIS, DIFFERENTIAL; NEURODEGENERATIVE DISEASES; PRION DISEASES; PRIONS;

EID: 0035902194     PISSN: 00284793     EISSN: None     Source Type: Journal    
DOI: 10.1056/NEJM200105173442006     Document Type: Article

References (119)

1. Development of the prion concept
2. Some speculations about prions, amyloid, and Alzheimer's disease
3. An epidemiological overview of amyotrophic lateral sclerosis, Parkinson's disease, and dementia of the Alzheimer type
4. Epidemiology of dementia and Alzheimer disease
5. Epidemiology of Parkinson's disease
6. Prevalence of Alzheimer's disease in a community population of older persons: Higher than previously reported
7. Prevalence of parkinsonian signs and associated mortality in a community population of older people
8. Prions
9. Scrapie prion rod formation in vitro requires both detergent extraction and limited proteolysis
10. Conversion of α-helices into β-sheets features in the formation of the scrapie prion proteins
11. NMR structure of the mouse prion protein domain PrP(121-231)
12. Solution structure of Syrian hamster prion protein rPrP(90-231)
13. Infectious and sporadic prion diseases
14. Prion diseases
15. Linkage of a prion protein mis-sense variant to Gerstmann-Sträussler syndrome
16. Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene
17. Analysis of the prion protein gene in thalamic dementia
18. Inherited prion disease with 144 base pair gene insertion. I. Genealogical and molecular studies
19. Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD)
20. The clinical characteristics of transmissible Creutzfeldt-Jakob disease
21. 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
22. A prion protein amino acid substitution in ataxic Gerstmann-Sträussler syndrome
23. Creutzfeldt-Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering
24. Creutzfeldt-Jakob disease in the United States, 1979-1994: Using national mortality data to assess the possible occurrence of variant cases
25. Clinical aspects of Creutzfeldt-Jakob disease
26. Creutzfeldt-Jakob disease: Focus among Libyan Jews in Israel
27. Neuropathology of prion diseases
28. Antibodies to a scrapie prion protein
29. Identification of prion amyloid filaments in scrapie-infected brain
30. Identification of a gene which controls the incubation period of some stratus of scrapie agent in mice
31. Biological evidence that scrapie agent has an independent genome
32. To what extent is strain variation evidence for an independent genome in the agent of the transmissible spongiform encephalopathies?
33. Changes in the localization of brain prion proteins during scrapie infection
34. Precise targeting of the pathology of the sialoglycoprotein, PrP, and vacuolar degeneration in mouse scrapie
35. Molecular biology of prion diseases
36. Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy
37. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity
38. Propagation of prion strains through specific conformers of the prion protein
39. Sc molecules with different conformations
40. Prion protein conformation in a patient with sporadic fatal insomnia
41. Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein
42. Pathologic conformations of prion proteins
43. Inherited prion diseases
44. Deaths from variant Creutzfeldt-Jakob disease
45. Tracking the human fallout from 'mad cow disease'
46. Predicted vCJD mortality in Great Britain
47. Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans
48. Possible person-to-person transmission of Creutzfeldt-Jakob disease
49. Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery
50. Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery
51. Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts - Japan, January 1979-May 1996
52. Progressive fatal dementia (Creutzfeldt-Jakob disease) in a patient who received homograft tissue for tympanic membrane closure
53. Creutzfeldt-Jakob disease in pituitary growth hormone recipients in the United States
54. Creutzfeldt-Jakob disease in a recipient of human pituitary-derived gonadotrophin
55. Codon 219 Lys allele of PRNP is not found in sporadic Creutzfeldt-Jakob disease
56. Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation
57. Dominant-negative inhibition of prion formation diminished by deletion mutagenesis of the prion protein
58. Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie
59. Association between natural scrapie and PrP genotype in a flock of Suffolk sheep in Scotland
60. Molecular genetics of Alzheimer disease
61. Intracellular APP processing and Aβ production in Alzheimer disease
62. Translating cell biology into therapeutic advances in Alzheimer's disease
63. Proteolytic processing and cell biological functions of the amyloid precursor protein
64. Nicastrin modulates presenilin-mediated notch/glp-1 signal transduction and βAPP processing
65. Association of apolipoprotein E allele ε4 with late-onset familial and sporadic Alzheimer's disease
66. Effects of age, sex, and ethnicity on the association between apolipoprotein E genotype and Alzheimer disease: A meta-analysis
67. Pathogenic implications of mutations in the tau gene in pallido-ponto-nigral degeneration and related neurodegenerative disorders linked to chromosome 17
68. Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17
69. Mutation in the tau gene in familial multiple system tauopathy with presenile dementia
70. Mutation-specific functional impairments in distract tau isoforms of hereditary FTDP-17
71. Tau protein isoforms, phosphorylation and role in neurodegenerative disorders
72. Frontal lobe degeneration of non-Alzheimer type revisited
73. Genetic evidence for the involvement of tau in progressive supranuclear palsy
74. Tau gene mutation in familial progressive subcortical gliosis
75. Genetics of Parkinson's disease
76. Parkinson disease in twins: An etiologic study
77. Mutation in the α-synuclein gene identified in families with Parkinson's disease
78. α-synuclein in Lewy bodies
79. Familial Parkinson disease gene product, parkin, is a ubiquitin-protein ligase
80. Oxidative damage linked to neurodegeneration by selective α-synuclein nitration in synucleinopathy lesions
81. A 10-year study of the incidence of and factors predicting dementia in Parkinson's disease
82. The Lewy body variant of Alzheimer's disease: A clinical and pathologic entity
83. α-synuclein in Lewy body disease and Alzheimer's disease
84. α-synuclein in filamentous inclusions of Lewy bodies from Parkinson's disease and dementia with Lewy bodies
85. Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: A review
86. Clinical features and diagnosis of amyotrophic lateral sclerosis
87. Epidemiology of motor-neuron diseases
88. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
89. Oxidation versus aggregation - How do SOD1 mutants cause ALS?
90. Molecular basis of the neurodegenerative disorders
91. Expanding our understanding of polyglutamine diseases through mouse models
92. Protein fate in neurodegenerative proteinopathies: Polyglutamine diseases join the (mis)fold
93. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
94. Glutamine repeats and neurodegeneration
95. Creutzfeldt-Jakob disease: Correlation of MRI and neuropathologic findings
96. Frontotemporal dementia and Alzheimer disease: Evaluation of cortical atrophy with automated hemispheric surface display generated with MR images
97. Detection of 14-3-3 protein in the cerebrospinal fluid supports the diagnosis of Creutzfeldt-Jakob disease
98. Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies
99. Alzheimer's soluble amyloid β is a normal component of human urine
100. Detection of single amyloid beta-protein aggregates in the cerebrospinal fluid of Alzheimer's patients by fluorescence correlation spectroscopy
101. Subacute spongiform encephalopathy - A subacute form of encephalopathy attributable to vascular dysfunction (spongiform cerebral atrophy)
102. Hashimoto's encephalitis as a differential diagnosis of Creutzfeldt-Jakob disease
103. Reversing neurodegeneration: A promise unfolds
104. Mimicking dominant negative inhibition of prion replication through structure-based drug design
105. Elimination of prions by branched polyamines and implications for therapeutics
106. Porphyrin and phthalocyanine antiscrapie compounds
107. Immunization with amyloid-beta attenuates Alzheimer-disease-like pathology in the PDAPP mouse
108. Success and problems of long-term levodopa therapy in Parkinson's disease
109. Narcolepsy: A neurodegenerative disease of the hypocretin system?
110. Localization of disinhibition-dementia-parkinsonism-amyotrophy complex to 17q21-22
111. Molecular and genetic aspects of multiple sclerosis
112. Identification of autoantibodies associated with myelin damage in multiple sclerosis
113. Heterogeneity of multiple sclerosis lesions: Implications for the pathogenesis of demyelination
114. Amyloidosis