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Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein

Human familial prion diseases are associated with mutations at 34 different prion protein (PrP) amino acid residues. However, it is unclear whether infectious prions are found in all cases. Mutant PrP itself may be neurotoxic, or alternatively, PrP mutation might predispose to spontaneous formation...

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Pubblicato in:Acta Neuropathol Commun
Autori principali: Race, Brent, Williams, Katie, Hughson, Andrew G., Jansen, Casper, Parchi, Piero, Rozemuller, Annemieke J. M., Chesebro, Bruce
Natura: Artigo
Lingua:Inglês
Pubblicazione: BioMed Central 2018
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC5819089/
https://ncbi.nlm.nih.gov/pubmed/29458424
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s40478-018-0516-2
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