Effectiveness of Anthracycline Against Experimental Prion Disease in Syrian Hamsters
- 16 May 1997
- journal article
- other
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 276 (5315) , 1119-1121
- https://doi.org/10.1126/science.276.5315.1119
Abstract
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.Keywords
This publication has 21 references indexed in Scilit:
- Amyloid fibrils in Gerstmann-Sträussler-Scheinker disease (Indiana and Swedish Kindreds) express only PrP peptides encoded by the mutant alleleCell, 1994
- BIOLOGY AND GENETICS OF PRION DISEASESAnnual Review of Microbiology, 1994
- A Neurotoxic Prion Protein Fragment Induces Rat Astroglial Proliferation and HypertrophyEuropean Journal of Neuroscience, 1994
- Neurotoxicity of a prion protein fragmentNature, 1993
- Potent Inhibition of Scrapie‐Associated PrP Accumulation by Congo RedJournal of Neurochemistry, 1992
- Amphotericin B treatment dissociates in vivo replication of the scrapie agent from PrP accumulationNature, 1992
- Amphotericin B Delays the Incubation Period of Scrapie in Intracerebrally Inoculated HamstersJournal of General Virology, 1987
- Dextran Sulphate 500 Delays and Prevents Mouse Scrapie by Impairment of Agent Replication in SpleenJournal of General Virology, 1984
- Scrapie prions aggregate to form amyloid-like birefringent rodsCell, 1983
- The antiviral compound HPA-23 can prevent scrapie when administered at the time of infectionArchiv für die gesamte Virusforschung, 1983