Transmissible spongiform encephalopathy: current and future therapeutic strategies

The importance of transmissible spongiform encephalopathies (TSE) and their treatment have increased since the outbreak of bovine spongiform encephalopathy in the UK and the cases of new variant Creutzfeldt-Jakob disease (CJD) that are considered to have arisen as a result. Treatments for clinical CJD have been unsuccessful, and in-vivo treatments of symptomatic animals inoculated with TSE have been similar. Polyene antifungal agents of iododoxurubicin have been successful in changing the incubation period of disease: the incidence of disease in inoculated animals has been decreased by sulphated polyglycosides and the presence of the prion form of the prion protein (PrPSC) in cellular cultures has been decreased by these and by Congo red. Theoretical methods have been suggested to prevent the development of disease in infected animals through the interaction of the normal form of the prion protein or by prevention of its alteration into PrPSC. Glycosidase inhibitors, glycoside transferase inhibitors, diazo dyes, modified polysulphonated polyglycosides, apoptosis inhibitors, and heat-shock proteins have all been suggested as methods of treatment of TSE infected animals. (C) 1998 Lippincott-Raven Publishers.