Variably Protease-Sensitive Prionopathy: A New Sporadic Disease of the Prion Protein

OBJECTIVE: The objective of the study is to report 2 new genotypic forms of protease-sensitive prionopathy (PSPr), a novel prion disease described in 2008, in 11 subjects all homozygous for valine at codon 129 of the prion protein (PrP) gene (129VV). The 2 new PSPr forms affect individuals who are e...

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Main Authors: Zou, Wen-Quan, Puoti, Gianfranco, Xiao, Xiangzhu, Yuan, Jue, Qing, Liuting, Cali, Ignazio, Shimoji, Miyuki, Langeveld, Jan P. M., Castellani, Rudy, Notari, Silvio, Crain, Barbara, Schmidt, Robert E., Geschwind, Michael, DeArmond, Stephen J., Cairns, Nigel J., Dickson, Dennis, Honig, Lawrence, Torres, Juan Maria, Mastrianni, James, Capellari, Sabina, Giaccone, Giorgio, Belay, Ermias D., Schonberger, Lawrence B., Cohen, Mark, Perry, George, Kong, Qingzhong, Parchi, Piero, Tagliavini, Fabrizio, Gambetti, Pierluigi
Formato: Artigo
Idioma:Inglês
Publicado em: 2010
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3032610/
https://ncbi.nlm.nih.gov/pubmed/20695009
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/ana.22094
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