Prion Proteins with Different Conformations Accumulate in Gerstmann-Sträussler-Scheinker Disease Caused by A117V and F198S Mutations

Gerstmann-Sträussler-Scheinker disease (GSS) is characterized by the accumulation of proteinase K (PK)-resistant prion protein fragments (PrP(sc)) of ∼7 to 15 kd in the brain. Purified GSS amyloid is composed primarily of ∼7-kd PrP peptides, whose N terminus corresponds to residues W(81) and G(88) t...

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Detalhes bibliográficos
Main Authors: Piccardo, Pedro, Liepnieks, Juris J., William, Albert, Dlouhy, Stephen R., Farlow, Martin R., Young, Katherine, Nochlin, David, Bird, Thomas D., Nixon, Randal R., Ball, Melvyn J., DeCarli, Charles, Bugiani, Orso, Tagliavini, Fabrizio, Benson, Merrill D., Ghetti, Bernardino
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Investigative Pathology 2001
Assuntos:
Regular Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC1891977/
https://ncbi.nlm.nih.gov/pubmed/11395398
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