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Prions: Protein Aggregation and Infectious Diseases
Aguzzi, Adriano; Calella, Anna Maria
Follow Physiological Reviews , Volume 89 (4): 1105
The American Physiological Society – Oct 1, 2009as infectious isolates that, when transmitted to identical hosts, exhibit distinct prion-disease phenotypes. The phenotypic traits may include distinct patterns of protein aggregate deposition, incubation times ...
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Aptamers against prion proteins and prions
Sabine, Gilch; Hermann, Schätzl
Follow Cellular and Molecular Life Sciences , Volume 66 (15)
Springer Journals – Aug 1, 2009are fatal neurodegenerative and infectious disorders of humans and animals, characterized by structural transition of the host-encoded cellular prion protein (PrP c ) into the aberrantly folded pathologic ...
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Structural Insights into Alternate Aggregated Prion Protein Forms
Polano, Maurizio; Bek, Alpan; Benetti, Federico; Lazzarino, Marco; Legname, Giuseppe
Follow Journal of Molecular Biology , Volume 393 (5)
Elsevier – Nov 13, 2009The conversion of the cellular form of the prion protein (PrP C ) to an abnormal, alternatively folded isoform (PrP Sc ) is the central event in prion diseases or transmissible spongiform ...
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Prion protein misfolding and disease
Moore, Roger A; Taubner, Lara M; Priola, Suzette A
Follow Current Opinion in Structural Biology , Volume 19 (1)
Elsevier – Feb 1, 2009diseases that involve the accumulation of an abnormally aggregated form of the normal host prion protein (PrP). They are unique among protein misfolding disorders in that they are transmissible and have ...
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Converting the prion protein: What makes the protein infectious
Baskakov, Ilia V.; Breydo, Leonid
Follow Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease , Volume 1772 (6)
Elsevier – Jun 1, 2007nature of prion infectivityThe prion hypothesis states that the infectious agent of prion diseases is an abnormally folded isoform of the prion protein (PrP Sc ) that replicates its abnormal ...
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Prions and the proteasome
Deriziotis, Pelagia; Tabrizi, Sarah J.
Follow Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease , Volume 1782 (12)
Elsevier – Dec 1, 2008because they are caused by the conformational re-arrangement of a normal host-encoded prion protein, PrP C , to an abnormal infectious isoform, PrP Sc . Currently the precise mechanism behind prion-mediated ...
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The hydrophobic core region governs mutant prion protein aggregation and intracellular retention
Emiliano Biasini*† 1 2 , Laura Tapella*†, Elena Restelli*†, Manuela Pozzoli*†, Tania Massignan*‡ and Roberto Chiesa*† 2
Follow Biochemical Journal , Volume 430 (3)
Portland Press – Sep 15, 2010in the pathogenesis of prion diseases.Key words: inherited prion disease, prion protein (PrP), protein aggregation, protein misfolding.Abbreviations used: BHK, baby hamster kidney; DAPI, 4′,6-diamidino-2 ...
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Peptide NMHRYPNQ of the Cellular Prion Protein (PrP C ) Inhibits Aggregation and Is a Potential Key for Understanding Prion–Prion Interactions
Rehders, Dirk; Claasen, Birgit; Redecke, Lars; Buschke, Alexander; Reibe, Caroline; Jehmlich, Nico; von Bergen, Martin; Betzel, Christian; Meyer, Bernd
Follow Journal of Molecular Biology , Volume 392 (1)
Elsevier – Sep 11, 2009degeneration of the grey matter and deposition of amyloid plaques leading to apoptosis of neural cells. According to the “protein-only theory,” the infectious agent of TSEs is the prion protein (PrP), which ...
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Prion: disease or relief?
Chernoff, Yury O.
Follow Nature Cell Biology , Volume 10 (9)
Nature Publishing Group (NPG) – Sep 1, 2008Prions cause infectious neurodegenerative diseases in mammals and control heritable traits in yeast . Most prions identified so far are self-polymerized amyloids that form highly ordered cross-β ...
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Size distribution dependence of prion aggregates infectivity
Calvez, Vincent; Lenuzza, Natacha; Oelz, Dietmar; Deslys, Jean-Philippe; Laurent, Pascal; Mouthon, Franck; Perthame, Benoît
Follow Mathematical Biosciences , Volume 217 (1)
Elsevier – Jan 1, 2009, scrapie in sheep and Creutzfeldt–Jakob disease (CJD) in human [1] . The infectious agents responsible for disease transmission, known as prions, present some unusual biological properties (as a high ...
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