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In Vitro Seeding Activity of Glycoform-Deficient Prions from Variably Protease-Sensitive Prionopathy and Familial CJD Associated with PrP(V180I) Mutation

Both sporadic variably protease-sensitive prionopathy (VPSPr) and familial Creutzfeldt-Jakob disease linked to the prion protein (PrP) V180I mutation (fCJD(V180I)) have been found to share a unique pathological prion protein (PrP(Sc)) that lacks the protease-resistant PrP(Sc) glycosylated at residue...

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Detalles Bibliográficos
Autores principales: Wang, Zerui, Yuan, Jue, Shen, Pingping, Abskharon, Romany, Lang, Yue, Dang, Johnny, Adornato, Alise, Xu, Ling, Chen, Jiafeng, Feng, Jiachun, Moudjou, Mohammed, Kitamoto, Tetsuyuki, Lee, Hyoung-gon, Kim, Yong-Sun, Langeveld, Jan, Appleby, Brian, Ma, Jiyan, Kong, Qingzhong, Petersen, Robert B., Zou, Wen-Quan, Cui, Li
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6614145/
https://www.ncbi.nlm.nih.gov/pubmed/30612334
http://dx.doi.org/10.1007/s12035-018-1459-0