CNS pathogenesis of prion diseases

British Medical Bulletin

Volumn 66, Issue , 2003, Pages 131-139

  Brandner, Sebastian ^a  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

CENTRAL NERVOUS SYSTEM; COGNITIVE DEFECT; DEGENERATIVE DISEASE; FATALITY; MOUSE; NERVOUS SYSTEM INJURY; NONHUMAN; PATHOGENESIS; PRION DISEASE; PRIORITY JOURNAL; REVIEW;

ANIMALS; CELL DEATH; CENTRAL NERVOUS SYSTEM; DISEASE SUSCEPTIBILITY; HUMANS; MICE; MODELS, ANIMAL; NEURONS; PRION DISEASES; PRIONS; PRPSC PROTEINS; SCRAPIE; SHEEP;

EID: 0141849467     PISSN: 00071420     EISSN: None     Source Type: Journal    
DOI: 10.1093/bmb/66.1.131     Document Type: Review

References (52)

1. Büeler HR, Fischer M, Lang Y et al. Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature 1992; 356: 577-82
2. Sailer A, Büeler H, Fischer M, Aguzzi A, Weissmann C. No propagation of prions in mice devoid of PrP. Cell 1994; 77: 967-8
3. Collinge J, Sidle KC, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996; 383: 685-90
4. Scott M, Foster D, Mirenda C et al. Transgenic mice expressing hamster prion protein produce species- specific scrapie infectivity and amyloid plaques. Cell 1989; 59: 847-57
5. 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 Sci USA 1994; 91: 9936-40
6. 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
7. Glatzel M, Heppner FL, Albers KM, Aguzzi A. Sympathetic innervation of lymphoreticular organs is rate limiting for prion neuroinvasion. Neuron 2001; 31: 25-34
8. Kaeser PS, Klein MA, Schwarz P, Aguzzi A. Efficient lymphoreticular prion propagation requires PrP(c) in stromal and hematopoietic cells. J Virol 2001; 75: 7097-106
9. Klein MA, Frigg R, Flechsig E et al. A crucial role for B cells in neuroinvasive scrapie. Nature 1997; 390: 687-90
10. Manson JC, Clarke AR, Hooper ML, Aitchison L, McConnell I, Hope J. 129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal. Mol Neurobiol 1994; 8: 121-7
11. Sakaguchi S, Katamine S, Nishida N et al. Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene. Nature 1996; 380: 528-31
12. Basler K, Oesch B, Scott M et al. Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene. Cell 1986; 46: 417-28
13. Tobler I, Gaus SE, Deboer T et al. Altered circadian activity rhythms and sleep in mice devoid of prion protein. Nature 1996; 380: 639-42
14. Collinge J, Whittington MA, Sidle KC et al. Prion protein is necessary for normal synaptic function. Nature 1994; 370: 295-7
15. Whittington MA, Sidle KC, 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
16. Rossi D, Cozzio A, Flechsig E, Klein MA, Aguzzi A, Weissmann C. Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brain. EMBO J 2001; 20: 1-9
17. Fischer M, Rülicke T, Raeber A et al. Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie. EMBO J 1996; 15: 1255-64
18. Prusiner SB, Scott M, Foster D et al. Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell 1990; 63: 673-86
19. Büeler HR, Aguzzi A, Sailer A et al. Mice devoid of PrP are resistant to scrapie. Cell 1993; 73: 1339-47
20. Weissmann C, Büeler H, Fischer M, Aguet M. Role of the PrP gene in transmissible spongiform encephalopathies. Intervirology 1993; 35: 164-75
21. Moore RC, Hope J, McBride PA et al. Mice with gene targeted prion protein alterations show that Prnp, Sinc and Prni are congruent. Nat Genet 1998; 18: 118-25
22. Brandner S, Isenmann S, Raeber A et al. Normal host prion protein necessary for scrapie-induced neurotoxicity. Nature 1996; 379: 339-43
23. Kretzschmar HA, Giese A, Brown DR et al. Cell death in prion disease. J Neural Transm Suppl 1997; 50: 191-210
24. Schätzl HM, Laszlo L, Holtzman DM et al. A hypothalamic neuronal cell line persistently infected with scrapie prions exhibits apoptosis. J Virol 1997; 71: 8821-31
25. Giese A, Groschup MH, Hess B, Kretzschmar HA. Neuronal cell death in scrapie-infected mice is due to apoptosis. Brain Pathol 1995; 5: 213-21
26. Gray F, Chretien F, Adle-Biassette H et al. Neuronal apoptosis in Creutzfeldt-Jakob disease. J Neuropathol Exp Neurol 1999; 58: 321-8
27. Milhavet O, McMahon HE, Rachidi W et al. Prion infection impairs the cellular response to oxidative stress. Proc Natl Acad Sci USA 2000; 97: 13937-42
28. Betmouni S, Perry VH, Gordon JL. Evidence for an early inflammatory response in the central nervous system of mice with scrapie. Neuroscience 1996; 74: 1-5
29. Wong BS, Liu T, Li R et al. Increased levels of oxidative stress markers detected in the brains of mice devoid of prion protein. J Neurochem 2001; 76: 565-72
30. Fraser JR. What is the basis of transmissible spongiform encephalopathy induced neurodegeneration and can it be repaired? Neuropathol Appl Neurobiol 2002; 28: 1-11
31. Park SK, Choi SI, Jin JK et al. Differential expression of Bax and Bcl-2 in the brains of hamsters infected with 263K scrapie agent [in Process Citation]. Neuroreport 2000; 11: 1677-82
32. Nicotera P. A route for prion neuroinvasion. Neuron 2001; 31: 345-8
33. Brown KL, Stewart K, Bruce ME, Fraser H. Severely combined immunodeficient (SCID) mice resist infection with bovine spongiform encephalopathy. J Gen Virol 1997; 78: 2707-10
34. 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: 183-9
35. Lasmézas CI, Deslys JP, Robain O et al. Transmission of the BSE agent to mice in the absence of detectable abnormal prion protein. Science 1997; 275: 402-5
36. Race R, Chesebro B. Scrapie infectivity found in resistant species [letter; see comments]. Nature 1998; 392: 770
37. Hill AF, Desbruslais M, Joiner S et al. The same prion strain causes vCJD and BSE [letter; see comments]. Nature 1997; 389: 448-50
38. Aguzzi A, Weissmann C. Spongiform encephalopathies. The prion's perplexing persistence. Nature 1998; 392: 763-4
39. Hill AF, Joiner S, Linehan J, Desbruslais M, Lantos PL, Collinge J. Species-barrier-independent prion replication in apparently resistant species. Proc Natl Acad Sci USA 2000; 97: 10248-53
40. Aguzzi A, Glatzel M, Montrasio F, Prinz M, Heppner FL. Interventional strategies against prion diseases. Nat Rev Neurosci 2001; 2: 745-9
41. Aguzzi A, Montrasio F, Kaeser PS. Prions: health scare and biological challenge. Nat Rev Mol Cell Biol 2001; 2: 118-26
42. Kimberlin RH, Walker CA. Pathogenesis of scrapie (strain 263K) in hamsters infected intracerebrally, intraperitoneally or intraocularly. J Gen Virol 1986; 67: 255-63
43. Kimberlin RH, Walker CA. Pathogenesis of experimental scrapie. Ciba Found Symp 1988; 135: 37-62
44. Fraser H. Neuronal spread of scrapie agent and targeting of lesions within the retino-tectal pathway. Nature 1982; 295: 149-50
45. Race R, Oldstone M, Chesebro B. Entry versus blockade of brain infection following oral or intraperitoneal scrapie administration: role of prion protein expression in peripheral nerves and spleen. J Virol 2000; 74: 828-33
46. Scott JR, Fraser H. Transport and targeting of scrapie infectivity and pathology in the optic nerve projections following intraocular infection. Prog Clin Biol Res 1989; 317: 645-52
47. Mallucci GR, Ratte S, Asante EA et al. Post-natal knockout of prion protein alters hippocampal CA1 properties, but does not result in neurodegeneration. EMBO J 2002; 21: 202-10
48. Krichevsky AM, Kosik KS. RNAi functions in cultured mammalian neurons. Proc Natl Acad Sci USA 2002; 99: 11926-9
49. Furth PA, St Onge L, Boger H et al. Temporal control of gene expression in transgenic mice by a tetracycline-responsive promoter. Proc Natl Acad Sci USA 1994; 91: 9302-6
50. Ornitz DM, Moreadith RW, Leder P. Binary system for regulating transgene expression in mice: targeting int-2 gene expression with yeast GAL4/UAS control elements. Proc Natl Acad Sci USA 1991; 88: 698-702
51. Tremblay P, Meiner Z, Galou M et al. Doxycycline control of prion protein transgene expression modulates prion disease in mice. Proc Natl Acad Sci USA 1998; 95: 12580-5
52. Shmerling D, Hegyi I, Fischer M et al. Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions. Cell 1998; 93: 203-14