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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書誌詳細
主要な著者: 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
フォーマット: Artigo
言語:Inglês
出版事項: American Society for Investigative Pathology 2001
主題:
Regular Article
オンライン･アクセス:https://ncbi.nlm.nih.gov/pmc/articles/PMC1891977/
https://ncbi.nlm.nih.gov/pubmed/11395398
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