Prion peptide uptake in microglial cells--the effect of naturally occurring autoantibodies against prion protein. by Yvonne Roettger, Inga Zerr, Richard Dodel, Jan-Philipp Bach

“…In prion disease, a profound microglial activation that precedes neurodegeneration has been observed in the CNS. …”
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Unexpected decrease of full-length prion protein in macaques inoculated with prion-contaminated blood products by Nina Jaffré, Jérôme Delmotte, Jacqueline Mikol, Jean-Philippe Deslys, Emmanuel Comoy

Subjects: “…prion…”
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Protease resistance of infectious prions is suppressed by removal of a single atom in the cellular prion protein. by Henning Leske, Simone Hornemann, Uli Simon Herrmann, Caihong Zhu, Paolo Dametto, Bei Li, Florent Laferriere, Magdalini Polymenidou, Pawel Pelczar, Regina Rose Reimann, Petra Schwarz, Elisabeth Jane Rushing, Kurt Wüthrich, Adriano Aguzzi

“…Detection of proteinase K-resistant prion protein (PrPSc) still represents the diagnostic gold standard for prion diseases in humans, sheep and cattle. …”
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Effects of solution chemistry and aging time on prion protein adsorption and replication of soil-bound prions. by Samuel E Saunders, Qi Yuan, Jason C Bartz, Shannon Bartelt-Hunt

“…Prion interactions with soil may play an important role in the transmission of chronic wasting disease (CWD) and scrapie. …”
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Detection limitations of prion seeding activities in blood samples from patients with sporadic prion disease by Toshiaki Nonaka, Yasushi Iwasaki, Hiroyuki Horiuchi, Katsuya Satoh

Subjects: “…Abnormal prion protein…”
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YEAST PRION PROTEIN Ure2p – A USEFUL MODEL FOR HUMAN PRION DISEASES. by Tatina T. Todorova, Gabriela St. Tsankova, Neli M. Ermenlieva

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Anti-prion drugs do not improve survival in novel knock-in models of inherited prion disease by Daniel J. Walsh, Judy R. Rees, Surabhi Mehra, Matthew E. C. Bourkas, Lech Kaczmarczyk, Erica Stuart, Walker S. Jackson, Joel C. Watts, Surachai Supattapone

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Anti-prion drugs do not improve survival in novel knock-in models of inherited prion disease. by Daniel J Walsh, Judy R Rees, Surabhi Mehra, Matthew E C Bourkas, Lech Kaczmarczyk, Erica Stuart, Walker S Jackson, Joel C Watts, Surachai Supattapone

“…Prion diseases uniquely manifest in three distinct forms: inherited, sporadic, and infectious. …”
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Neuronal Ndst1 depletion accelerates prion protein clearance and slows neurodegeneration in prion infection. by Patricia Aguilar-Calvo, Adela Malik, Daniel R Sandoval, Christopher Barback, Christina D Orrù, Heidi G Standke, Olivia R Thomas, Chrissa A Dwyer, Donald P Pizzo, Jaidev Bapat, Katrin Soldau, Ryotaro Ogawa, Mckenzie B Riley, K Peter R Nilsson, Allison Kraus, Byron Caughey, Jeffrey J Iliff, David R Vera, Jeffrey D Esko, Christina J Sigurdson

“…Select prion diseases are characterized by widespread cerebral plaque-like deposits of amyloid fibrils enriched in heparan sulfate (HS), a abundant extracellular matrix component. …”
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Interaction networks of prion, prionogenic and prion-like proteins in budding yeast, and their role in gene regulation. by Djamel Harbi, Paul M Harrison

“…Prions are transmissible, propagating alternative states of proteins. …”
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Prion induction involves an ancient system for the sequestration of aggregated proteins and heritable changes in prion fragmentation by Tyedmers, Jens, Treusch, Sebastian, Dong, Jijun, McCaffery, J. Michael, Bevis, Brooke J., Lindquist, Susan

“…To do so, we used a visually tractable prion model consisting of the Sup35 prion domain fused to GFP (PrD-GFP) and overexpressed it to achieve induction in many cells simultaneously. …”
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THE NATIONAL PRION MONITORING COHORT: A LONGITUDINAL OBSERVATIONAL STUDY OF UK HUMAN PRION INFECTION by Mead, S, Gajulapalli, D, Walker, S, Wroe, S, Rudge, P, Collinge, J

Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein by Brent Race, Katie Williams, Andrew G. Hughson, Casper Jansen, Piero Parchi, Annemieke J. M. Rozemuller, Bruce Chesebro

“…Abstract Human familial prion diseases are associated with mutations at 34 different prion protein (PrP) amino acid residues. …”
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Peroxiredoxin 6 promotes upregulation of the prion protein (PrP) in neuronal cells of prion-infected mice by Wagner Wibke, Reuter Andreas, Hüller Petra, Löwer Johannes, Wessler Silja

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Oral Ingestion of Synthetically Generated Recombinant Prion Is Sufficient to Cause Prion Disease in Wild-Type Mice by Chenhua Pan, Junwei Yang, Xiangyi Zhang, Ying Chen, Shunxiong Wei, Guohua Yu, Yi-Hsuan Pan, Jiyan Ma, Chonggang Yuan

Subjects: “…prion…”
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Lesion of the olfactory epithelium accelerates prion neuroinvasion and disease onset when prion replication is restricted to neurons. by Jenna Crowell, James A Wiley, Richard A Bessen

“…Natural prion diseases of ruminants are moderately contagious and while the gastrointestinal tract is the primary site of prion agent entry, other mucosae may be entry sites in a subset of infections. …”
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Epigenetic Changes in Prion and Prion-like Neurodegenerative Diseases: Recent Advances, Potential as Biomarkers, and Future Perspectives by Adelaida Hernaiz, Janne Markus Toivonen, Rosa Bolea, Inmaculada Martín-Burriel

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Novel polymorphisms in the prion protein gene (PRNP) and stability of the resultant prion protein in different horse breeds by Diego Sola, Rody Artigas, Diego R. Mediano, Pilar Zaragoza, Juan José Badiola, Inmaculada Martín-Burriel, Cristina Acín

Subjects: “…Prion disease…”
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Insight From Animals Resistant to Prion Diseases: Deciphering the Genotype – Morphotype – Phenotype Code for the Prion Protein by Ryan Myers, Alessandro Cembran, Pedro Fernandez-Funez

Subjects: “…prion disease…”
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Systemic delivery of siRNA down regulates brain prion protein and ameliorates neuropathology in prion disorder. by Sylvain Lehmann, Aroa Relano-Gines, Sarah Resina, Elsa Brillaud, Danielle Casanova, Charles Vincent, Claire Hamela, Sophie Poupeau, Mathieu Laffont, Audrey Gabelle, Constance Delaby, Maxime Belondrade, Jacques-Damien Arnaud, Maria-Teresa Alvarez, Jean-Claude Maurel, Patrick Maurel, Carole Crozet

“…These results suggest that a new therapeutic scheme based an innovative delivery system of PrP-siRNA can be envisioned in prion disorders.…”
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