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Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS.
PLoS Biol. 2020 Jun 9;18(6):e3000725. doi: 10.1371/journal.pbio.3000725. eCollection 2020 Jun.
PLoS Biol. 2020.
PMID: 32516343
Free PMC article.
Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins.
Asante EA, Gowland I, Grimshaw A, Linehan JM, Smidak M, Houghton R, Osiguwa O, Tomlinson A, Joiner S, Brandner S, Wadsworth JDF, Collinge J.
Asante EA, et al. Among authors: smidak m.
J Gen Virol. 2009 Mar;90(Pt 3):546-558. doi: 10.1099/vir.0.007930-0.
J Gen Virol. 2009.
PMID: 19218199
Free PMC article.
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Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein.
Asante EA, Linehan JM, Smidak M, Tomlinson A, Grimshaw A, Jeelani A, Jakubcova T, Hamdan S, Powell C, Brandner S, Wadsworth JD, Collinge J.
Asante EA, et al. Among authors: smidak m.
PLoS Pathog. 2013;9(9):e1003643. doi: 10.1371/journal.ppat.1003643. Epub 2013 Sep 26.
PLoS Pathog. 2013.
PMID: 24086135
Free PMC article.
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A naturally occurring variant of the human prion protein completely prevents prion disease.
Asante EA, Smidak M, Grimshaw A, Houghton R, Tomlinson A, Jeelani A, Jakubcova T, Hamdan S, Richard-Londt A, Linehan JM, Brandner S, Alpers M, Whitfield J, Mead S, Wadsworth JD, Collinge J.
Asante EA, et al. Among authors: smidak m.
Nature. 2015 Jun 25;522(7557):478-81. doi: 10.1038/nature14510. Epub 2015 Jun 10.
Nature. 2015.
PMID: 26061765
Free PMC article.
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Transmission Properties of Human PrP 102L Prions Challenge the Relevance of Mouse Models of GSS.
Asante EA, Grimshaw A, Smidak M, Jakubcova T, Tomlinson A, Jeelani A, Hamdan S, Powell C, Joiner S, Linehan JM, Brandner S, Wadsworth JD, Collinge J.
Asante EA, et al. Among authors: smidak m.
PLoS Pathog. 2015 Jul 2;11(7):e1004953. doi: 10.1371/journal.ppat.1004953. eCollection 2015 Jul.
PLoS Pathog. 2015.
PMID: 26135918
Free PMC article.
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Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice.
Ghazi-Noori S, Froud KE, Mizielinska S, Powell C, Smidak M, Fernandez de Marco M, O'Malley C, Farmer M, Parkinson N, Fisher EM, Asante EA, Brandner S, Collinge J, Isaacs AM.
Ghazi-Noori S, et al. Among authors: smidak m.
Brain. 2012 Mar;135(Pt 3):819-32. doi: 10.1093/brain/aws006.
Brain. 2012.
PMID: 22366797
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