Results 31 to 40 of about 7,097 (179)

Pathologic prion protein is specifically recognized in situ by a novel PrP conformational antibody

Neurobiology of Disease, 2006
Prion diseases are characterized by the accumulation in the brain of abnormal conformers (PrPSc) of the cellular prion protein (PrPC).PrPSc immunohistochemistry, currently based on antibodies non-distinguishing between PrPC and PrPSc, requires pre ...
Gianluca Moroncini, Michela Mangieri, Michela Morbin, Giulia Mazzoleni, Bernardino Ghetti, Armando Gabrielli, Robert Anthony Williamson, Giorgio Giaccone, Fabrizio Tagliavini   +8 more
doaj   +1 more source

Deficiency in ST6GAL1, one of the two α2,6-sialyltransferases, has only a minor effect on the pathogenesis of prion disease

Frontiers in Molecular Biosciences, 2022
Prion diseases are a group of fatal neurodegenerative diseases caused by misfolding of the normal cellular form of the prion protein or PrPC, into a disease-associated self-replicating state or PrPSc.
Natallia Makarava, Natallia Makarava, Elizaveta Katorcha, Elizaveta Katorcha, Jennifer Chen-Yu Chang, Jennifer Chen-Yu Chang, Joseph T. Y. Lau, Ilia V. Baskakov, Ilia V. Baskakov   +8 more
doaj   +1 more source

Dynamics and genetics of PrPSc placental accumulation in sheep

Journal of General Virology, 2007
Placentae from scrapie-affected ewes are an important source of contamination. This study confirmed that scrapie-incubating ewes bearing susceptible genotypes could produce both abnormal prion protein (PrPSc)-positive and -negative placentae, depending only on the PRP genotype of the fetus.
Lacroux, Caroline, Corbière, Fabien, Tabouret, Guillaume, Lugan, Séverine, Costes, Pierrette, Mathey, Jacinthe, Delmas, Jean-Marc, Weisbecker, Jean Louis, Foucras, Gilles, Cassard, Hervé, Elsen, Jean Michel, Schelcher, Francois, Andréoletti, Olivier   +12 more
openaire   +4 more sources

The protean prion protein.

PLoS Biology, 2020
The prion protein, PrP, can adopt at least 2 conformations, the overwhelmingly prevalent cellular conformation (PrPC) and the scrapie conformation (PrPSc).
Jesús R Requena
doaj   +1 more source

Double-Edge Sword of Sustained ROCK Activation in Prion Diseases through Neuritogenesis Defects and Prion Accumulation. [PDF]

PLoS Pathogens, 2015
In prion diseases, synapse dysfunction, axon retraction and loss of neuronal polarity precede neuronal death. The mechanisms driving such polarization defects, however, remain unclear.
Aurélie Alleaume-Butaux, Simon Nicot, Mathéa Pietri, Anne Baudry, Caroline Dakowski, Philippe Tixador, Hector Ardila-Osorio, Anne-Marie Haeberlé, Yannick Bailly, Jean-Michel Peyrin, Jean-Michel Peyrin, Jean-Marie Launay, Odile Kellermann, Benoit Schneider   +13 more
doaj   +1 more source

PrPSc Incorporation to Cells Requires Endogenous Glycosaminoglycan Expression [PDF]

Journal of Biological Chemistry, 2005
Many lines of evidence suggest an interaction between glycosaminoglycans (GAGs) and the PrP proteins as well as a possible role for GAGs in prion disease pathogenesis. In this work, we sought to determine whether the PrP-GAG interaction affects the incorporation of PrP(Sc) (the scrapie isoform of PrP) to normal cells.
Nuha, Hijazi, Zehavit, Kariv-Inbal, Maria, Gasset, Ruth, Gabizon   +3 more
openaire   +2 more sources

Combating ageing beyond the cell: Emerging roles of extracellular proteostasis

The FEBS Journal, EarlyView.
Ageing challenges the body's ability to maintain a stable and functional proteome, leading to protein damage and aggregation both inside and outside cells. This review focuses on the less well understood mechanisms of extracellular protein quality control and how they become disrupted in ageing, particularly in neurodegenerative diseases.
Siddharth R. Venkatesh, Ivan Gallotta, Bolanle F. Olabiyi, Yoon Hee Choi, Sarah Inglesfield, Janet R. Kumita, Collin Y. Ewald, Della C. David   +7 more
wiley   +1 more source

Preserving prion strain identity upon replication of prions in vitro using recombinant prion protein

Acta Neuropathologica Communications, 2018
Last decade witnessed an enormous progress in generating authentic infectious prions or PrPSc in vitro using recombinant prion protein (rPrP). Previous work established that rPrP that lacks posttranslational modification is able to support replication of
Natallia Makarava, Regina Savtchenko, Peter Lasch, Michael Beekes, Ilia V. Baskakov   +4 more
doaj   +1 more source

A novel method for preclinical detection of PrPSc in blood

Journal of General Virology, 2010
In this study, we demonstrate that a moderate amount of protein misfolding cyclic amplification (PMCA) coupled to a novel surround optical fibre immunoassay (SOFIA) detection scheme can be used to detect the disease-associated form of the prion protein (PrP(Sc)) in protease-untreated plasma from preclinical and clinical scrapie sheep, and white-tailed ...
Richard, Rubenstein, Binggong, Chang, Perry, Gray, Martin, Piltch, Marie S, Bulgin, Sharon, Sorensen-Melson, Michael W, Miller   +6 more
openaire   +2 more sources

Aptamer‐Targeted PrPC Drives Colorectal Cancer Metastasis via a LYN‐STAT3 Complex and Enables Liquid Biopsy Detection

Advanced Science, Volume 13, Issue 45, 13 August 2026.
The aptamer WHY‐3E identifies PrPC as a CRC driver. Stabilized by USP18, endocytosed PrPC forms a LYN/STAT3 complex, upregulating MSN transcription to promote metastasis. Crucially, WHY‐3E sensitively detects PrPC‐positive circulating exosomes, establishing a robust theoretical foundation for non‐invasive clinical diagnostics.
Chunlin Wang, Hongyu Wu, Chaojing Zheng, Hao Zhang, Xiaoqiu Wu, Jiaqi Wang, Zewen Chang, Jun Xiang, Yunxiao Liu, Chenkai Zhang, Yuliuming Wang, Hao Jiang, Yuchen Zhong, Jun Luo, Ying Chen, NaNa Zhang, Weiyuan Zhang, Ziming Yuan, ChaoXia Zou, Weihong Tan, Meng Wang, Hanqing Hu, Tao Bing, Guiyu Wang   +23 more
wiley   +1 more source