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Publication : Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies.

First Author  Heppner FL Year  2001
Journal  Science Volume  294
Issue  5540 Pages  178-82
PubMed ID  11546838 Mgi Jnum  J:127850
Mgi Id  MGI:3765141 Doi  10.1126/science.1063093
Citation  Heppner FL, et al. (2001) Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies. Science 294(5540):178-82
abstractText  Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy are initiated by extracerebral exposure to prions. Although prion transmission from extracerebral sites to the brain represents a potential target for prophylaxis, attempts at vaccination have been limited by the poor immunogenicity of prion proteins. To circumvent this, we expressed an anti-prion protein (anti-PrP) mu chain in Prnp(o/o) mice. Transgenic mice developed sustained anti-PrP titers, which were not suppressed by introduction of Prnp+ alleles. Transgene expression prevented pathogenesis of prions introduced by intraperitoneal injection in the spleen and brain. Expression of endogenous PrP (PrP(C)) in the spleen and brain was unaffected, suggesting that immunity was responsible for protection. This indicates the feasibility of immunological inhibition of prion disease in vivo.
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