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Review: Contribution of transgenic models to understanding human prion disease

Content Provider	Scilit
Author	Wadsworth, J. D. F. Asante, E. A. Collinge, J.
Copyright Year	2010
Description	Journal: Neuropathology and applied neurobiology J. D. F. Wadsworth, E. A. Asante and J. Collinge (2010) Neuropathology and Applied Neurobiology36, 576–597Contribution of transgenic models to understanding human prion disease Transgenic mice expressing human prion protein in the absence of endogenous mouse prion protein faithfully replicate human prions. These models reproduce all of the key features of human disease, including long clinically silent incubation periods prior to fatal neurodegeneration with neuropathological phenotypes that mirror human prion strain diversity. Critical contributions to our understanding of human prion disease pathogenesis and aetiology have only been possible through the use of transgenic mice. These models have provided the basis for the conformational selection model of prion transmission barriers and have causally linked bovine spongiform encephalopathy with variant Creutzfeldt‐Jakob disease. In the future these models will be essential for evaluating newly identified potentially zoonotic prion strains, for validating effective methods of prion decontamination and for developing effective therapeutic treatments for human prion disease.
Related Links	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3017745/pdf
Ending Page	597
Page Count	22
Starting Page	576
e-ISSN	13652990
DOI	10.1111/j.1365-2990.2010.01129.x
Journal	Neuropathology and applied neurobiology
Issue Number	7
Volume Number	36
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2010-09-28
Access Restriction	Open
Subject Keyword	Journal: Neuropathology and applied neurobiology Creutzfeldt‐jakob Disease Fatal Familial Insomnia Gerstmann‐sträussler‐scheinker Disease Variant Creutzfeldt‐jakob Disease
Content Type	Text
Resource Type	Article

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