Prion diseases are transmissible neurodegenerative conditions characterized by the accumulation of protease-resistant forms of the prion protein (PrP), termed PrPres, in the brain. Insoluble PrPres tends to aggregate into amyloid fibrils. The anthracycline 4'-iodo-4'-deoxy- doxorubicin (IDX) binds to amyloid fibrils and induces amyloid resorption in patients with systemic amyloidosis. To test IDX in an experimental model of prion disease, Syrian hamsters were inoculated intracerebrally either with scrapie-infected brain homogenate or with infected homogenate coincubated with IDX. In IDX-treated hamsters, clinical signs of disease were delayed and survival time was prolonged. Neuropathological examination showed a parallel delay in the appearance of brain changes and in the accumulation of PrPres and PrP amyloid.
Effectiveness of anthracyclines against experimental prion disease in Syrian hamsters / F. Tagliavini, R.A. McArthur, B. Canciani, G. Giaccone, M. Porro, M. Bugiani, P.M.J. Lievens, O. Bugiani, E. Peri, P. Dall’Ara, M. Rocchi, G. Poli, G. Forloni, T. Bandiera, M. Varasi, A. Suarato, P. Cassutti, M.A. Cervini, J. Lansen, M. Salmona, C. Post. - In: SCIENCE. - ISSN 0036-8075. - 276:5315(1997), pp. 1119-1122.
|Titolo:||Effectiveness of anthracyclines against experimental prion disease in Syrian hamsters|
|Settore Scientifico Disciplinare:||Settore VET/05 - Malattie Infettive degli Animali Domestici|
Settore MED/05 - Patologia Clinica
Settore MED/26 - Neurologia
|Data di pubblicazione:||1997|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1126/science.276.5315.1119|
|Appare nelle tipologie:||01 - Articolo su periodico|