Transmissible spongiform encephalopathies

New England Journal of Medicine

Volumn 337, Issue 25, 1997, Pages 1821-1828

  Haywood, Anne M ^a  

^a UNIVERSITY OF ROCHESTER MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

PRION PROTEIN;

BOVINE SPONGIFORM ENCEPHALOPATHY; CREUTZFELDT JAKOB DISEASE; HUMAN; INCUBATION TIME; KURU; NEUROPATHOLOGY; NONHUMAN; PATHOGENESIS; PRION DISEASE; PRIORITY JOURNAL; REVIEW; SPECIES DIFFERENCE;

ANIMALS; HUMANS; MOLECULAR BIOLOGY; PRION DISEASES; PRIONS;

EID: 0031457078     PISSN: 00284793     EISSN: None     Source Type: Journal    
DOI: 10.1056/NEJM199712183372508     Document Type: Review

References (69)

1. Wells GAH, Scott AC, Johnson CT, et al. A novel progressive spongiform cncephalopathy in cattle. Vet Roc 1987;121:419-20.
2. Will RU, Ironside JW, Zcidlcr M, et al. A new variant of Crcutzfeldt-Jakob disease in the UK. Lancet 1996;347:921-5.
3. Cuillé J, Chclle P-L. La maladie dite tremblante du mouton est elle inoculable? Comptes Rendus Acad Sci 1936;203:1552 4.
4. Chandler RL. Enccphalopathy in mice produced by inoculation with scrapic brain material. Lancet 1961;1:1378 9.
5. Rohwer RG. The scrapic agent: "a vims by any other name." Curr lop Microbiol Immunol 1991;172:195-232.
6. Chesebru H, Fields BN. Transmissible spongiform encephalopathies: a brief introduction. In: Fields BN, Kiiipc DM, Howlev PM, eds. fields virology. 3rd ed. Vol. 2. Philadelphia: Lippincott-Raven, 1996:2845-9.
7. Schreuder BEC. General aspects of transmissible spongiform encepha lopathies and hypotheses about the agents. Vet Q 1993;15:167-74.
8. Griffith IS. Self-replication und scrapie. Nature 1967;215:1043-4.
9. OcArniond SJ, 1'rusiner SB. Etiology and pathogcncsis of prion diseases. Am J Pathol 1995;I46:785-811.
10. Prusiner SB. Molecular biology and pathogenesis of prion diseases. Trends Biochem Sci 1996;2 1:482 7.
11. Weissmann C. The ninth Datta lecture: molecular biology of transmissible spongiform eneephalopathies. FEBS Lett 1996;389:3-11.
12. Mamiclidis L. Dementias, neurodegeneration, and viral mechanisms of disease from the perspective of human transmissible eneephalopathies. Ann N Y Acad Sci 1994;724:259-81.
13. Idem. The dimensions of Creutztcldt-Iakob disease. Transfusion 1994;34:915-28.
14. Haywood AM. Patterns of persistent viral infections. N Engl I Med 1986:315:939 48.
15. Dickinson AG, Outram GW. Genetic aspects of unconventional virus infections: the basis of the virino hypothesis. Ciba Found Symp 1988;! 35:3-83.
16. Kimberlin RH, Walker ('A. Pathogenesis of experimental scrapie. Ciba Found Symp 1988;l35:37-62.
17. Idem. Pathogenesis of scrapie in mice after intragastrie infection. Virus Res 1989:12:213-20.
18. Kimberlin RH, Cole S, Walker ('A. Temporary and permanent modifications to a single strain of mouse scrapie on transmission to rats and hamsters. I Gen Virol 1987:68:1875-81.
19. Bruce ME. Scrapie strain variation and mutation. Br Med Bull 1993;49:822-38.
20. Bruce ME, McConnell 1, Eraser H, Dickinson AG. The disease characteristics of different strains of scrapie in Sine congenic mouse lines: implications tor the nature of the agent and host control of pathogenesis. J Gen Virol 1991;72:595-603.
21. Hunter N, Dann JC, Bennett AD, Somerville RA, McConnell I, Hope I. Are Sinc and the PrP gene congruent: E,vidence from PrP gene analysis in Sine congenic mice. J Gen Virol 1992;73:2751-5.
22. Merz PA, Rohwer RG, Kascsak R, et al. Infection-specific particle from the unconventional slow virus diseases. Science 1984;225:437-40.
23. Bolton DC, McKinley MP, Prusiner SB. Identification of a protein that purities with the scrapie prion. Science 1982;218:1309-11.
24. Diringer H, Gelderblom H, Hilmert H, Özel M, Edelbluth C, Kimberlin RH. Scrapie infcctivity, fibrils and low molecular weight protein. Nature 1983;306:476-8.
25. Chcscbro B, Race R, Wehrly K, et al. Identification of scrapie prion protein-specific mRNA in scrapie-infectcd and uninfected brain. Nature 1985:315:331-3.
26. Ocsch B, Wcstaway D, Wälchli M, et al. A cellular gene encodes scrap ic PrP 27-30 protein. Cell 1985;40:735-46.
27. Kstibeiro IP. Multiple roles for PrP in the prion diseases. Trends Neurosei 1996;19:257-8.
28. Stahl N, Baldwin MA, Teplow DB, et al. Structural studies of the serapie prion protein using mass spectrometry and amino acid sequencing. Biochemistry 1993;32:1991-2002.
29. Pan K-M, Baldwin M, N'guycn J, et al. Conversion of α-hclices into β-sheets features in the formation of the scrapic prion proteins. Proc Natl Acad Sci U S A 1993;90:10962-6.
30. Safar I. Spectroscopic conformational studies ot prion protein isotbrms and the mechanism of transformation. Semin Virol 1996;7:207-14.
31. Cauglley B, Raymond GI. The scrapie-associated form of PrP is made from a cell surface precursor that is both protease- and phospholipase-sensitive. I Biol Chem 1991:266:18217-23.
32. Beekes M, Baldanf E, Diringer H. Sequential appearance and accumulation of palhognomonic markers in the central nervous system of hamsters orally infected with scrapie. I Gen Virol 1996;77:1925-34.
33. I.asme/as CI, Deslys J-P, Robain O, et al. Transmission of the BSE agent to mice in the absence of detectable abnormal prion protein. Science 1997;275:402-5.
34. Biieler H, Fischer M, Lang Y, et al. Normal development and behaviour ot mice lacking the neuronal cell-surface PrP protein. Nature 1992;356:577-82.
35. Tobler I, Gaus SE, Debocr l, et al. Altered circadian activity rhythms and sleep in mice devoid ot prion protein. Nature 1996;380:639-42.
36. Sakaguchi S, Katamine S, Nishida X, et al. Loss ot ccrebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene. Nature 1996;380:28-31.
37. Biieler H, Aguzzi A, Sailer A, et al. Mice devoid of PrP are resistant to scrapie. Cell 1993;73:1339-47
38. Prusiner SB, Scott M, Foster D, et al. Transgenetic studies implicate interactions between homologous PrP isotbrms in serapie prion replicalion. Cell I990;63:673-86.
39. 'Idling GC, Scott M, Mastrianni I, 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.
40. Budka H, Aguzzi A, Brown P, et al. Ncuropathological diagnostic criteria tor Creutzf'dt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases). Brain Pathol 1995;5:459-66.
41. Hsich G, Kenney K, Gibbs CJ Jr, Lee KH, Harrington MG. The 14 3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform cncephalopathies. N Engl J Med 1996;335:924-30.
42. Zerr I, Bodemer M, Racker S, et al. Cerebrospinal fluid concentration of neuron-specific enolase in diagnosis of Creutzfeldt-Jakob disease. Lancet 1995;345:1609 10.
43. Brown P, Coker-Vann M, Pomeroy K, et al. Diagnosis of CreutzfeldtJakob disease by Western blot identification of marker protein in human brain tissue. N Engl I Med 1986;314:547-51.
44. Parehi P, Castcllani R, Capellari S, et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt Jakob disease. Aim Neurol 1996;39:767 78.
45. Collingc J, Sidle KCL, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of'new variant' C)D. Nature 1996;383:685-90.
46. Williams ES, Young S. Spongiform encephalopathies in Cervidae. Rev Sci Tech 1992;! 1:551-67.
47. McKenzie D, Bartz JC, Marsh RF. Transmissible mink encephalopathy. Semin Virol 1996;7:201 -6.
48. Sehreuder BEC. Animal spongiform encephaloparhies - an update. I. Scrapie and lesser-known animal spongiform encephalopathies. Vet Q 1994;16:174-81.
49. Idem. Animal spongiform encephalopathies - an update. II. Bovine spongiform encephalopathy (BSE). Vet Q 1994;16:182-92.
50. Wells GAH, Wilcsmith JW. The neuropathology and epidemiology of bovine spongiform encephalopathy. Brain Pathol 1995;5:91-103. [Erratum, Brain Pathol 1995;5:200.]
51. Bruce ME, Chree A, McConnell I, Foster J, Pearson G, Fraser H. Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier. Philos Trans R Soc Loud B Biol Sci 1994;343:405-11.
52. Kimberlin RH. Speculations on the origin of BSE and the epidemiology of CJD. In: Gibbs CJ Jr, ed. Bovine spongiform encephalopathy. New York: Springer Verlag, 1996:155-75.
53. Petersen RB, Tabaton M, Chen SG, et al. Familial progressive subcor-tical gliosis: presence of prions and linkage to chromosome 17. Neurology 1995;45:1062-7. [Erratum, Neurology 1995;45:1430.]
54. Sarkozi E, Askanas V, Engel WK. Abnormal accumulation of prion protein mRNA in muscle fibers of patients with sporadic inclusion-body myositis and hereditary inclusion-body myopathy. Am J Pathol 1994;145: 1280-4.
55. Gajdusek DC. Unconventional viruses and the origin and disappear ance of kuru. Science 1977;197:943-60.
56. Coflinge J, Palmer MS, Dryden AJ. Genetic predisposition to iatrogenic Crcutzfeldt-Jakob disease. Lancet 1991;337:1441-2.
57. Brown P. The phenotypic expression of different mutations in transmissible human spongiform encephalopathy. Rev Neurol (Paris) 1992;148:17-27.
58. Ghetti B, Piccardo P, Frangione B, et al. Prion protein hereditary amy loidosis: parenchymal and vascular. Semin Virol 1996;7:189-200.
59. Medori R, Tritschler H-J, LeBlanc 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;326:444-9.
60. Brown P, Preece MA, Will RG. "Friendly fire" in medicine: hormones, homografts, and Creutzfcldt Jakob disease. Lancet 1992;340:24-7.
61. Brown P, Gibbs CJ Jr, Rodgers-Johnson P, et al. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of ex perimentally transmitted disease. Ann Neurol 1994;35:513-29.
62. Ironside JW. Creutzfeldt-Jakob disease. Brain Pathol 1996;6:379-88.
63. Richardson EP Jr, Masters CL. The nosology of'Creutzfeldt-Jakob disease and conditions related to the accumulation of PrP''"1 in the nervous system. Brain Pathol 1995;5:33-41.
64. Brown P. EEG findings in Greutzfeldt-Jakob disease. JAMA 1993;269:168.
65. Collee JG, Bradley R. BSE: a decade on. Lancet 1997;349:715 21.
66. Verdrager J. Creutztcldt Jakob disease. Lancet I996;347:1704.
67. Lasmézas CI, Deslys J-P, Demalmay R, et al. BSE transmission to macaques. Nature 1996;381:743-4.
68. 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.
69. Cousens SN, Vynnycky E, Zeidler M, Will RG, Smith PG. Predicting the CJD epidemic in humans. Nature 1997;385:197-8.