EFFECTIVENESS OF ANTHRACYCLINE AGAINST EXPERIMENTAL PRION DISEASE IN SYRIAN-HAMSTERS

Prion diseases are transmissible neurodegenerative conditions characte rized by the accumulation of protease-resistant forms of the prion pro tein (PrP), termed PrPres, in the brain. Insoluble PrPres tends to agg regate into amyloid fibrils. The anthracycline 4'-iodo-4'-deoxy-doxoru bicin (IDX) binds to amyloid fibrils and induces amyloid resorption in patients with systemic amyloidosis. To test IDX in an experimental mo del of prion disease, Syrian hamsters were inoculated intracerebrally either with scrapie-infected brain homogenate or with infected homogen ate coincubated with IDX. In IDX-treated hamsters, clinical signs of d isease were delayed and survival time was prolonged. Neuropathological examination showed a parallel delay in the appearance of brain change s and in the accumulation of PrPres and PrP amyloid.