Prion diseases in man

Journal of Pathology

Volumn 186, Issue 3, 1998, Pages 227-234

  Ironside, James W ^a,b  

^a UNIVERSITY OF EDINBURGH   (United Kingdom)

^b WESTERN GENERAL HOSPITAL   (United Kingdom)

Author keywords

Bovine spongiform encephalopathy; Creutzfeldt Jakob disease; Immunocytochemistry; Neuropathology; Prion; Spongiform encephalopathy

Indexed keywords

CELL SURFACE PROTEIN; PRION PROTEIN;

AUTOPSY; BOVINE SPONGIFORM ENCEPHALOPATHY; CREUTZFELDT JAKOB DISEASE; DIAGNOSTIC ACCURACY; DISEASE CLASSIFICATION; FAMILIAL DISEASE; FOOD CHAIN; HUMAN; NEUROPATHOLOGY; NONHUMAN; PRION DISEASE; PRIORITY JOURNAL; PROTEIN FOLDING; REVIEW; SCRAPIE; UNITED KINGDOM;

ANIMALS; CATTLE; HUMANS; MUTATION; PRION DISEASES; PRIONS; PROTEIN STRUCTURE, SECONDARY;

EID: 0031729399     PISSN: 00223417     EISSN: None     Source Type: Journal    
DOI: 10.1002/(SICI)1096-9896(1998110)186:3<227::AID-PATH174>3.0.CO;2-3     Document Type: Review

References (85)

1. Bastian FO (ed.). Creutzfeldt-Jakob Disease and Other Transmissible Human Spongiform Encephalopathies. St Louis: Moseby Year Book, 1991.
2. Cuille J, Chelle P-L. La malade dite temblente du mouton est-elle inocu-lable? C R Acad Sci 1936; 203: 1552-1554.
3. Kimberlin RH. Scrapie. Br Vet J 1981; 137: 105-112.
4. Brown P, Cathala F, Raubertas RF, et al. The epidemiology of Creutzfeldt-Jakob disease: conclusion of a 15-year investigation in France and review of the world literature. Neurology 1987; 37: 295-304.
5. Adams DH, Caspary EA, Field EJ. Susceptibility of scrapie agent to ionising radiation. Nature 1969; 221: 90-91.
6. Pattison I. Resistance of the scrapie agent to formalin. J Comp Pathol 1965; 75: 159-164.
7. Griffiths J. Self replication and scrapie. Nature 1967; 215: 1043-1044.
8. Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science 1982; 216: 136-144.
9. Prusiner SB. Biology and genetics of prion disease. Annu Rev Microbiol 1994; 48: 655-686.
10. Tobler I, Gaus SE, Deboer T, et al. Altered circadian activity rhythms and sleep in mice devoid of prion proteins. Nature 1996; 380: 639-642.
11. Collinge J, Whittington M, Sidle KCL, et al. Prion protein is necessary for normal synaptic function. Nature 1994; 370: 295-297.
12. Brown DR, Qin K, Herms JW, et al. The cellular prion protein binds copper in vivo. Nature 1997; 390: 684-687.
13. Sailer A, Bueller H, Fischer M, Aguzzi A, Weissmann C. No propagation of prions in mice devoid of PrP. Cell 1994; 77: 967-968.
14. Liao Y-CJ, Lebo RV, Clawson GA, Smuckler EA. Human prion protein cDNA: molecular cloning, chromosomal mapping, and biological implications. Science 1986; 233: 364-367.
15. Shyng SL, Huber MT, Harris DA. A prion protein cycle between the cell surface and endocytic compartment in cultured neuroblastoma cells. J Biol Chem 1993; 268: 15 922-15 928.
16. Huang Z, Prusiner SB, Cohen FE. Scrapie prions: a three-dimensional model of an infectious fragment. Fold Des 1996; 1: 13.
17. Peretz D, Williamson RA, Matsunaga Y, et al. A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform. J Mol Biol 1997; 273: 614-622.
18. 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 1997; 94: 10 069-10 074.
19. Raymond GJ, Hope J, Kocisko DA, et al. Molecular assessment of the potential transmissibilities of BSE and scrapie to humans. Nature 1997: 388: 228-229.
20. Collinge J, Sidle KC. Meads J, Ironside JW, Hill AF. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996: 383: 685-690.
21. DeArmond SJ. Alzheimer's disease and Creutzfeldt-Jakob disease: overlap of pathogenic mechanisms. Curr Opin Neurol 1993; 6: 872-881.
22. Creutzfeldt HG. Uber eine eigenartige herdformige Erkrankung des Zentralnervensystems. Z Gesamte Neurol Psychiatr 1920; 57: 1-18.
23. Jakob A. Uber eigenartige Erkrankungen des Zentralnervensystems mit bemerkenswertem anatomichen Befunde (Spastische Pseudosklerose-Encephalomyelopathie mit disseminierten Degenerations-herden). Z Gesamte Neurol Psychiatr 1921; 64: 147-228.
24. CJd in the nervous system. Brain Pathol 1995; 5: 33-41.
25. Brown P, Cervenakova L, Boellaard JW, et al. Identification of a PRNP gene mutation in Jakob's original Creutzfeldt-Jakob disease family. Lancet 1994; 344: 130-131.
26. Gajdusek DC, Zigas V. Degenerative disease of the central nervous system in New Guinea: the endemic occurrence of 'kuru' in the native population. N Engl J Med 1957; 257: 974-978.
27. Klitzman RL, Alpers MP, Gadjdusek DC. The natural incubation period of kuru and the episodes of transmission in three clusters of patients. Neuro-epidemiology 1984; 3: 3-20.
28. Alpers M. Epidemiology and clinical aspects of kuru. In: Prusiner SB. McKinley MP, eds. Prions: Novel Infectious Agents Causing Scrapie and Creutzfeldt-Jakob Disease. San Diego: Academic Press, 1989; 451-467.
29. Gajdusek DC, Gibbs CJ, Alpers M. Experimental transmission of a kuru-like syndrome to chimpanzees. Nature 1966; 209: 794-796.
30. Gibbs CJ, Gajdusek DC, Asher DM. Creutzfeldt-Jakob disease (spongiform encephalopathy) transmission to the chimpanzee. Science 1968; 161: 388-389.
31. Gerstmann J, Straussler E, Scheinker I. Uber eine eigenartige hereditar-familiäre Erkrankung des Zentralnervensystems. Zugleich ein Beitrag zur frag des vorzeitigen lakalen Alterns. Z Neurol 1936; 154: 736-762.
32. Masters CL, Gajdusek DC, Gibbs CJ. Creutzfeldt-Jakob disease: virus isolations from the Gerstmann-Straussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced encephalopathies. Brain 1981; 104: 559-588.
33. Hsiao K, Baker HF, Crow TJ, et al. Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome. Nature 1989; 338: 342-345.
34. Goldfarb LG, Brown P, Cervenakova L, Gajdusek DC. Molecular genetic studies of Creutzfeldt-Jakob disease. Mol Neurobiol 1994; 8: 89-97.
35. Ironside JW, Bell JE. The 'high-risk' neuropathological autopsy in AIDS and Creutzfeldt-Jakob disease: principles and practice. Neuropathol Appl Neurobiol 1996; 22: 388-393.
36. Budkha H, Aguzzi A, Brown P, et al. Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases). Brain Pathol 1995; 5: 459-466.
37. Kretzschmar HA, Ironside JW, DeArmond SJ, Tateishi J. Diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 53: 913-920.
38. Masters CL, Richardson EP. Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). The nature and progression of spongiform change. Brain 1978; 101: 333-334.
39. Hansen LA, Masliah E, Terry RD, Mirra SS. A neuropathological subset of Alzheimer's disease with concomitant Lewy body disease and spongiform change. Acta Neuropathol 1989; 78: 194-201.
40. Bell JE, Ironside JW. Neuropathology of spongiform encephalopathies in humans. Br Med Bull 1993; 43: 738-777.
41. Ironside JW. Review: Creutzfeldt-Jakob disease. Brain Pathol 1996; 144: 379-388.
42. Klatzo I, Gajdusek DC, Zigas V. Pathology of kuru. Lab Invest 1959; 8: 799-847.
43. Bell JE, Gentleman SM, Ironside JW, et al. Prion protein immunocytochemistry - U.K. five centre consensus report. Neuropathol Appl Neurobiol 1997; 23: 25-35.
44. Will RG. Surveillance of prion diseases in humans. In: Baker HF, Ridley RM, eds. Prion Diseases. Totawa, NJ: Humana Press, 1996; 119-137.
45. Delasnerie-Laupretre N, Poser S, Pocchiari M. Incidence of Creutzfeldt-Jakob disease in Europe. Lancet 1995; 346: 898.
46. Parchi P. Castellani R. Cappellari S. et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39: 669-680.
47. MacDonald ST, Sutherland K, Ironside JW. Prion protein genotype and pathological phenotype studies in sporadic Creutzfeldt-Jakob disease. Neuropathol Appl Neurobiol 1996; 22: 285-292.
48. Gambetti P, Parchi P, Petersen PB, Chen SG, Lugaresi E. Fatal familial insomnia and familial Creutzfeldt-Jakob disease: clinical, pathological and molecular features. Brain Pathol 1996; 6: 127-145.
49. Palmer MS, Dryden AJ, Hughes JT, Collinge J. Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature 1991; 352: 340-342.
50. Collinge J, Palmer MS, Dryden AJ. Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease. Lancet 1991; 337: 1441-1442.
51. Collinge J, Palmer M. Human prion diseases. In: Collinge J, Palmer M, eds. Prion Disease. Oxford: Oxford University Press, 1997; 18-57.
52. Miller DC. Creutzfeldt-Jakob disease in histopathology technicians. N Engl J Med 1988; 318: 853.
53. Gorman DG, Benson DF, Vogel DG, Vinters HV. Creutzfeldt-Jakob disease in a pathologist. Neurology 1992; 42: 463.
54. Van Duijn CM. Delasnerie-Laupretre N, Masullo C, et al. Case control study of risk factors of Creutzfeldt-Jakob disease in Europe during 1993-95. Lancet 1998: 351: 1081-1085.
55. Brown P, Cathala F, Castaigne P, Gajdusek DC. Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol 1986; 20: 597-602.
56. Bortone E, Bettoni L, Giori C, Terzano MG, Trabattoni GR, Mancia D. Reliability of EEG in the diagnosis of Creutzfeldt-Jakob disease. Electroencephalogr Clin Neurophysiol 1994; 90: 323-330.
57. Finkenstaedt M, Szudra A, Zerr I, et al. MR imaging of Creutzfeldt-Jakob disease. Radiology 1996; 199: 793-798.
58. Kitamoto T, Tateishi J. Immunohistochemical confirmation of Creutzfeldt-Jakob disease with a long clinical course with amyloid plaque core antibodies. Am J Pathol 1988; 131: 435-443.
59. Shibuya S, Higuchi J, Shin R-W, Tateishi J, Kitamoto T. Protective prion protein polymorphisms against sporadic Creutzfeldt-Jakob disease. Lancet 1998; 351: 419.
60. Collinge J, Owen F, Poulter M, et al. Prion dementia without characteristic pathology. Lancet 1990; 336: 7-9.
61. Hainfellner JA, Branter-Inthaler S, Cervenakova L. The original Gerstmann-Straussler-Scheinker family of Austria: divergent clinicopathological phenotypes but constant PrP genotype. Brain Pathol 1995; 5: 201-211.
62. Amouyel P, Vidai O, Launay JM, Laplanche JL. The apolipoprotein E alleles as major susceptibility factors for Creutzfeldt-Jakob disease. Lancet 1994; 344: 1315-1318.
63. Nakagawa Y, Kitamoto T, Furukawa H, Ogomori K, Tateishi J. Apolipoprotein E in Creutzfeldt-Jakob disease. Lancet 1995; 345: 66-69.
64. Duffy P, Wolf J. Collins G, et al. Possible person-to-person transmission of Creutzfeldt-Jakob disease. N Engl J Med 1974; 290: 692-693.
65. . Powell-Jackson J. Weller R, Kennedy P, et al. Creutzfeidt-Jakob disease following human growth hormone administration. Lancet 1985; 2: 244-246.
66. Gibbs CJ, Joy A, Heffner R, et al. Clinical and pathological features and laboratory confirmation of Creutzfeldt-Jakob disease in a recipient of pituitary-derived human growth hormone. N Engl J Med 1985; 313: 734-739.
67. Brown P, Preece MA, Will RG. 'Friendly fire' in medicine: hormones homografts and Creutzfeldt-Jakob disease. Lancet 1992; 340: 24-27.
68. Deslys J-P, Jaegly A, d'Aignaux JH, et al. Genotype at codon 129 and susceptibility to Creutzfeldt-Jakob disease. Lancet 1998; 351: 1251.
69. Martinez-Lage JF, Poza M, Sola J, et al. Accidental transmission of Creutzfeldt-Jakob disease by dural cadaveric grafts. J Neurol Neurosurg Psychiatry 1994; 57: 1091-1094.
70. Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the U.K. Lancet 1996; 347: 921-925.
71. Ironside JW, Sutherland ?, Bell JE, et al. A new variant of Creutzfeldt-Jakob disease: neuropathological and clinical features. Cold Spring Harbor Symp Quant Biol 1996; 61: 523-530.
72. 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.
73. Zeidler M, Stewart G, Cousens SN, Estibeiro K, Will RG. Codon 129 genotype and new variant CJD. Lancet 1997; 350: 668.
74. 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.
75. Hill AF, Zeidler M. Ironside JW, Collinge J. Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy. Lancet 1997; 349: 99-100.
76. van Keulen LJM, Schreuder BEC, Meloen RH, et al. Immunohistochemical detection of prion protein in lymphoid tissues of sheep with natural scrapie. J Clin Microbiol 1996; 34: 1228-1231.
77. Ludlam C. New variant Creutzfeldt-Jakob disease and treatment of haemophilia. Lancet 1997; 349: 1704.
78. Esmonde TFG, Will RG, Slattery JM, et al. Creutzfeldt-Jakob disease and blood transfusion. Lancet 1993; 341: 205-207.
79. Cousens SN, Vynnyncky E, Zeidler M, et al. Predicting the CJD epidemic in humans. Nature 1997; 385: 197-198.
80. Advisory Committee on Dangerous Pathogens. Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection. London: The Stationery Office, 1998.
81. Hsich G, Kenney K, Gibbs CJ, Lee KH, Harrington MG. The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. N Engl J Med 1996; 335: 924-930.
82. Will RG, Zeidler M, Brown P, Harrington M, Lee KH, Kennedy KL. Cerebrospinal fluid test for new variant Creutzfeldt-Jakob disease. Lancet 1996; 348: 955.
83. Caughey B, Ernst D, Race RE. Congo red inhibition of scrapie agent replication. J Virol 1993; 67: 6270-6272.
84. Caughey B. Scrapie associated PrP accumulation and its prevention: insights from cell culture. Br Med Bull 1993; 49: 860-872.
85. Prusiner SB. Cell biology and transgenic models of prion diseases. In: Collinge J, Palmer MS, eds. Prion Diseases. New York: Oxford University Press, 1997; 130-162.