Biopolym. Cell. 1999; 15(2):93-102.
Reviews
Bovine Spongiform encephalopathy (BSE) — mad cow disease
- University College London
Gower Str., London, UK, WC1E 6BT
Abstract
The background to the epidemic of BSE among cattle in the United Kingdom is described as are the possible origins of the disease and its relationship to scrapie in sheep. Although the epidemic in cattle is virtually over there is evidence of the transmission of the infectious agent to humans to produce a new variant of Creutzfeld-Jacob disease. The current status of our understanding of the molecular biology of the infectious agent is described as is the evidence in support of the protein, only, prion, hypothesis. Study of the glycoforms of the prions supports the view that BSE has been transmitted to humans.
Full text: (PDF, in Ukrainian)
References
[1]
Hunter G. JX Scrapie and Mad Cow Disease. New York: Vantage press, 1993.
[2]
Prions Prions Prions. Ed. S. B. Prusiner. Heidelberg: Springer, 1996.
[3]
Update on BSC. London: Royal Soc., 1997.
[4]
Smith C, Collinge J. Molecular pathology of prion diseases. Essays Biochem. 1995;29:157-74.
[5]
Donnelly CA, Ghani AC, Ferguson NM, Anderson RM. Recent trends in the BSE epidemic. Nature. 1997;389(6654):903.
[6]
Klein MA, Frigg R, Flechsig E, Raeber AJ, Kalinke U, Bluethmann H, Bootz F, Suter M, Zinkernagel RM, Aguzzi A. A crucial role for B cells in neuroinvasive scrapie. Nature. 1997;390(6661):687-90. 1982;216(4542):136-44.
[7]
Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science. 1982;216(4542):136-44.
[8]
Bueler H, Aguzzi A, Sailer A, Greiner RA, Autenried P, Aguet M, Weissmann C. Mice devoid of PrP are resistant to scrapie. Cell. 1993;73(7):1339-47. ttp://
[9]
Riek R, Hornemann S, Wider G, Glockshuber R, Wuthrich K. NMR characterization of the full-length recombinant murine prion protein, mPrP(23-231). FEBS Lett. 1997;413(2):282-8.
[10]
Raymond GJ, Hope J, Kocisko DA, Priola SA, Raymond LD, Bossers A, Ironside J, Will RG, Chen SG, Petersen RB, Gambetti P, Rubenstein R, Smits MA, Lansbury PT Jr, Caughey B. Molecular assessment of the potential transmissibilities of BSE and scrapie to humans. Nature. 1997;388(6639):285-8.
[11]
Paushkin SV, Kushnirov VV, Smirnov VN, Ter-Avanesyan MD. In vitro propagation of the prion-like state of yeast Sup35 protein. Science. 1997;277(5324):381-3.
[13]
Hegde RS, Mastrianni JA, Scott MR, DeFea KA, Tremblay P, Torchia M, DeArmond SJ, Prusiner SB, Lingappa VR. A transmembrane form of the prion protein in neurodegenerative disease. Science. 1998;279(5352):827-34.
[14]
Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG, Farlow M, Dickson DW, Sima AA, Trojanowski JQ, Petersen RB, Gambetti P. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol. 1996 ;39(6):767-78.
[15]
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(6602):685-90.
[16]
Hill AF, Desbruslais M, Joiner S, Sidle KC, Gowland I, Collinge J, Doey LJ, Lantos P. The same prion strain causes vCJD and BSE. Nature. 1997;389(6650):448-50, 526.
[17]
Aguzzi A, Weissmann C. Spongiform encephalopathies: a suspicious signature. Nature. 1996;383(6602):666-7.
[18]
Lasmezas CI, Deslys JP, Demaimay R, Adjou KT, Lamoury F, Dormont D, Robain O, Ironside J, Hauw JJ. BSE transmission to macaques. Nature. 1996;381(6585):743-4.
[19]
Bruce ME, Will RG, Ironside JW, McConnell I, Drummond D, Suttie A, McCardle L, Chree A, Hope J, Birkett C, Cousens S, Fraser H, Bostock CJ. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature. 1997;389(6650):498-501.
[20]
Korth C, Stierli B, Streit P, Moser M, Schaller O, Fischer R, Schulz-Schaeffer W, Kretzschmar H, Raeber A, Braun U, Ehrensperger F, Hornemann S, Glockshuber R, Riek R, Billeter M, Wuthrich K, Oesch B. Prion (PrPSc)-specific epitope defined by a monoclonal antibody. Nature. 1997;390(6655):74-7.
[21]
Lasmezas CI, Deslys JP, Robain O, Jaegly A, Beringue V, Peyrin JM, Fournier JG, Hauw JJ, Rossier J, Dormont D. Transmission of the BSE agent to mice in the absence of detectable abnormal prion protein. Science. 1997;275(5298):402-5.
[24]
Transmissible spongiform encephalopaties: a summary of present. Knowledge and Research. London: HMSO, 1994. 50 p.