TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES, HYPOTHESES AND FOOD SAFETY- AN OVERVIEW

The transmissible spongiform encephalopathies (TSEs) (in both humans a nd animals) have been reviewed with the principal focus on bovine spon giform encephalopathy (BSE), its recent outbreak amongst cattle in the UK ('mad cow disease') and its sequelae. The possible reasons for thi s outbreak are discussed as well as a number of hypotheses reviewed fo r TSEs (e.g. prions, organophosphates, etc.) and current measures atte ndant with food safety and surveillance taken in the European Union. I t is generally accepted that the combined weight of all the evidence t o date supports the conclusion that the new rare but lethal variant Cr eutzfeldt-Jacob Disease (vCJD) is the human counterpart of the aetiolo gical agent BSE and that eating meat from the infected animals is prob ably to blame for 24 deaths (23 in the UK and one in France) to date f rom vCJD. Considerable evidence from biochemical, immunologic, patholo gic and genetic studies strongly suggests that Prp(sc) is the major co mponent of the transmissible prion particle responsible for the rare f atal brain TSE diseases. (C) 1998 Elsevier Science B.V. All rights res erved.