Bovine Spongiform Encephalopathy (BSE) – Mad Cow Disease

The background to the epidemic of bovine spongiform encephalopathy (BSE) among cattle in the United Kingdom is described as the possible origin 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 Creutzfeldt-Jakob 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. OOOOOOOOOOOOOOOOO Received: March 29, 1998 Dr. Peter Campbell Department of Biochemistry and Molecular Biology University College London Gower Street London, WC1E 6BT (UK) ABC Fax + 41 61 306 12 34 E-Mail [email protected] www.karger.com © 1998 S. Karger AG, Basel 1011–7571/98/0073–0172$15.00/0 Accessible online at: http://BioMedNet.com/karger Introduction Until 1985 there were probably not many biochemists in the UK who were more than vaguely aware that sheep tended to suffer from a degenerative disease known as ‘scrapie’. Moreover, since the disease had existed in the UK for some 200 years and no one had suggested that it could be transmitted to humans the subject was hardly a prominent one for research. Nevertheless, the British Government did continue to finance research at a modest level in order to unravel the nature of the infective agent, often described as a ‘slow virus’, and similar work was pursued in other countries, especially the USA. All this changed following the report in 1985 from a farm in Kent, that they had a cow suffering from what is now called ‘mad cow disease’. After a delay of about a year before the full significance of the finding became apparent, the situation rapidly changed. We have witnessed a biological problem of mammoth proportions which has many implications in terms of the aetiology of the disease and the nutrition of animals and humans. Although most of the troubles have been centred in the UK there have been a small D ow nl oa de d by : 54 .1 91 .4 0. 80 9 /1 6/ 20 17 4 :0 3: 26 P M