Prion diseases: An update

Current Opinion in Psychiatry

Volumn 11, Issue 3, 1998, Pages 299-303

  Raeber, Alex J ^a   Aguzzi, Adriano ^a  

^a UNIVERSITY HOSPITAL ZURICH   (Switzerland)

Author keywords

[No Author keywords available]

Indexed keywords

PRION PROTEIN;

BOVINE SPONGIFORM ENCEPHALOPATHY; CREUTZFELDT JAKOB DISEASE; DIETARY INTAKE; FOOD CONTAMINATION; HUMAN; MOLECULAR BIOLOGY; PHENOTYPE; PRION DISEASE; REVIEW;

EID: 0031894051     PISSN: 09517367     EISSN: None     Source Type: Journal    
DOI: 10.1097/00001504-199805000-00011     Document Type: Review

References (45)

1. Prusiner SB. Prion diseases and the BSE crisis. Science 1997; 278:245-251.
2. Aguzzi A, Weissmann C. Prion research: the next frontiers. Nature 1997; 389:795-798.
3. Prusiner SB, Scott M, Foster D, Pan KM, Groth D, Mirenda C, et al. Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell 1990; 63:673-686.
4. Scott MR, Safar J, Telling G, Nguyen O, Groth D, Torchia M, et al. Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice. Proc Natl Acad Sci U S A 1997; 94:14279-14284.
5. Aguzzi A, Weissmann C. Sleepless in Bologna: transmission of fatal familial insomnia. Trends Microbiol 1996; 4:129-131.
6. Büeler HR, Aguzzi A, Sailer A, Greiner RA, Autenried P, Aguet M, Weissmann C. Mice devoid of PrP are resistant to scrapie. Cell 1993; 73:1339-1347.
7. C) required for scrapie spread within the central nervous system. Proc Natl Acad Sci U S A 1996; 93:13148-13151.
8. Sc would require one polypeptide to adopt an infinite number of conformations, an argument frequently expressed by opponents of the 'protein only' hypothesis. In this work, two independently derived strains converge upon multiple serial transmissions in transgenic mice and hamsters. This provides compelling evidence for a limited number of prion strains and supports the notion that prion strain diversity is contained entirely within PrP.
9. Kocisko DA, Come JH, Priola SA, Chesebro B, Raymond GJ, Lansbury PT, Caughey B. Cell-free formation of protease-resistant prion protein. Nature 1994: 370:471-474.
10. Bessen RA, Kocisko DA, Raymond GJ, Nandan S, Lansbury PT, Caughey B. Non-genetic propagation of strain-specific properties of scrapie prion protein. Nature 1995; 375:698-700,
11. Kocisko DA, Priola SA, Raymond GJ, Chesebro B, Lansbury PT Jr, Caughey B. Species specificity in the cell-free conversion of prion protein to protease-resistant forms: a model for the scrapie species barrier. Proc Natl Acad Sci U S A 1995; 92:3923-3927.
12. C has been reported. This structure reveals a highly flexible tail harbouring the octapeptide repeats with possible physiological implications (see [15•]).
13. C.
14. Hornshaw MP, McDermott JR, Candy JM Copper binding to the N-terminal tandem repeat regions of mammalian and avian prion protein. Biochem Biophys Res Commun 1995; 207:621-629.
15. O O mice.
16. Brown DR, Schulz-Schaeffer WJ, Schmidt B, Kretzschmar HA. Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activity. Exp Neurol 1997; 146:104-112.
17. Yehiely F, Bamborough P, Da Costa M, Perry BJ, Tninakaran G, Cohen FE, et al. Identification of candidate proteins binding to prion protein. Neurobiol Dis 1997; 3:339-355.
18. Rieger R, Edenhofer F, Lasmezas CI, Weiss S. The human 37-kDalaminin receptor precursor interacts with the prion protein in eukaryotic cells. Nature Med 1997; 3:1383-1388
19. Martins VR, Graner E, Garcia-Abreu J, de Souza SJ, Mercadante AF, Veiga SS, et al. Complementary hydropathy identifies a cellular prion protein receptor. Nature Med 1997; 3:1376-1382.
20. Samaia HB, Mari JJ, Vallada HP, Moura RP, Simpson AJ, Brentani RR. A prion-linked psychiatric disorder [scientific correspondence]. Nature 1997; 390:241.
21. Weissmann C, Aguzzi A. Bovine spongiform encephalopathy and early onset variant Creutzfeldt-Jakob disease. Curr Opin Neurobiol 1997; 7:695-700.
22. Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996; 347:921-925.
23. Zeidler M, Stewart GE, Barraclough CR, Bateman DE, Bates D, Burn DJ, et al. New variant Creutzfeldt-Jakob disease: neurological features and diagnostic tests. Lancet 1997; 350:903-907.
24. Lasmezas Cl, Deslys JP, Demaimay R, Adjou KT, Lamoury F, Dormont D, et al. BSE transmission to macaques. Nature 1996; 381:743-744.
25. Bruce ME, Will RG, Ironside JW, McConnell I, Drummond D, Suttie A, et al. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 1997; 389:498-501. An elegant study is presented that uses classical strain typing experiments to demonstrate that BSE and variant CJD prions are identical but clearly distinct from sporadic CJD prions.
26. Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39:767-778.
27. Bessen RA, Marsh RF, Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy. J Virol 1994; 68:7859-7868.
28. 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; 383:685-690.
29. Hill AF, Desbruslais M, Joiner S, Sidle KC, Gowland I, Collinge J, et al. The same prion strain causes vCJD and BSE. Nature 1997; 389:448-450. The authors demonstrate that BSE and variant CJD (but not sporadic CJD) prions are indistinguishable in that they display identical 'fingerprint' pattern on Western blots upon transmission to mice.
30. Telling GC, Parchi P, DeArmond SJ, Cortelli P, Montagna P, Gabizon R, et al. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science 1996; 274:2079-2082.
31. Hill AF, Will RG, Ironside J, Collinge J, Type of prion protein in UK farmers with Creutzfeldt-Jakob disease [letter]. Lancet 1997; 350:188.
32. Somerville RA, Chong A, Mulqueen OU, Birkett CR, Wood SC, Hope J. Biochemical typing of scrapie strains [scientific correspondence]. Nature 1997; 386:564.
33. Sc.
34. Raymond GJ, Hope J, Kocisko DA, Priola SA, Raymond LD, Bossers A, et al. Molecular assessment of the potential transmissibilities of BSE and scrapie to humans. Nature 1997; 388:285-288.
35. Telling GC, Scott M, Mastrianni J, Gabizon R, Torchia M, Cohen FE, 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.
36. Race RE, Priola SA, Bessen RA, Ernst D, Dockter J, Rall GF, et al. Neuron-specific expression of a hamster prion protein minigene in transgenic mice induces susceptibility to hamster scrapie agent. Neuron 1995; 15:1183-1191.
37. Brandner S, Isenmann S, Kühne G, Aguzzi A. Identification of the end stage of scrapie using infected neural grafts. Brain Pathol 1998; 8:19-27.
38. Raeber AJ, Race RE, Brandner S, Priola SA, Sailer A, Bessen RA, et al. Astrocyte-specific expression of hamster prion protein (PrP) renders PrP knockout mice susceptible to hamster scrapie. EMBO J 1997; 16:6057-6065.
39. Eklund CM, Kennedy RC, Hadlow WJ. Pathogenesis of scrapie virus infection in the mouse. J Infect Dis 1967; 117:15-22.
40. Hill AF, Zeidler M, Ironside J, Collinge J. Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy [letter]. Lancet 1997; 349:99.
41. Kitamoto T, MuramotoT, Mohri S, Dohura K, Tateishi J. Abnormal isoform of prion protein accumulates in follicular dendritic cells in mice with Creutzfeldt-Jakob disease. J Virol 1991; 65:6292-6295.
42. Blattler T, Brandner S, Raeber AJ, Klein MA, VoigtlanderT, Weissmann C. Aguzzi A. PrP-expressing tissue required for transfer of scrapie infectivity from spleen to brain. Nature 1997; 389:69-73.
43. Klein M, Frigg R, Flechsig E, Raeber AJ, Kalinke U, Bluethmann U, et al. A crucial role for B-cells in neuroinvasive scrapie. Nature 1997; 390:687-690.
44. Cousens SN, Vynnycky E, Zeidler M, Will RG, Smith PG. Predicting the CJD epidemic in humans. Nature 1997; 385:197-198.
45. Aguzzi A, Collinge J, Post-exposure prophylaxis after accidental prion inoculation. Lancet 1997; 350:1519-1520.