Results 121 to 130 of about 73,750 (253)

Cystatin F is a biomarker of prion pathogenesis in mice.

PLoS ONE, 2017
Misfolding of the cellular prion protein (PrPC) into the scrapie prion protein (PrPSc) results in progressive, fatal, transmissible neurodegenerative conditions termed prion diseases.
Mario Nuvolone, Nicolas Schmid, Gino Miele, Silvia Sorce, Rita Moos, Christian Schori, Roger R Beerli, Monika Bauer, Philippe Saudan, Klaus Dietmeier, Ingolf Lachmann, Michael Linnebank, Roland Martin, Ulf Kallweit, Veronika Kana, Elisabeth J Rushing, Herbert Budka, Adriano Aguzzi   +17 more
doaj   +1 more source

Analysis of the hippocampal proteome in ME7 prion disease reveals a predominant astrocytic signature and highlights the brain-restricted production of clusterin in chronic neurodegeneration

, 2013
Prion diseases are characterized by accumulation of misfolded protein, gliosis, synaptic dysfunction, and ultimately neuronal loss. This sequence, mirroring key features of Alzheimer disease, is modeled well in ME7 prion disease.
Asuni, Ayodeji A, Bailey, Joanne, Gray, Bryony, O'Connor, Vincent, Perry, V Hugh, Skipp, Paul   +5 more
core   +1 more source

Prions and Prion Diseases [PDF]

Laboratory Medicine, 1999
Prion diseases (transmissible encephalopathies) are progressive neurodegenerative diseases of animals and humans caused by a unique agent, the prion. These diseases have recently been in the news because of the emergence of bovine spongiform encephalopathy (BSE; mad cow disease) and variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom.
openaire   +1 more source

Chronic Wasting Disease management responses in North America: A public policy analysis

Wildlife Society Bulletin, EarlyView.
In this study we use the Multiple Streams Framework from public policy theory to assess the responses of wildlife management agencies in states and provinces with CWD‐positive cases in the United States and Canada to alleviate public concerns and manage the spread of this disease.
Kelly H. Dunning, Iree Wheeler, Catherine A. Cummings, Stephen S. Ditchkoff, Braxton McCoy, Daniel Morris, Philip Reichert, Vincent Rivers, Travis E. Stoakley, Temple Stoellinger, Tarissa Spoonhunter, Janna R. Willoughby, Sarah Zohdy   +12 more
wiley   +1 more source

Prions, prion-like prionoids, and neurodegenerative disordersVacancy

Annals of Indian Academy of Neurology, 2016
Prion diseases or transmissible spongiform encephalopathies are fatal neurodegenerative diseases characterized by the aggregation and deposition of the misfolded prion protein in the brain.
Ashok Verma
doaj   +1 more source

Epilepsia partialis continua as the presenting manifestation of Creutzfeldt–Jakob disease: A video‐polygraphic clinical vignette

Epileptic Disorders, EarlyView.
Roberta Cutellè, Angelo Pascarella, Ada Santoro, Vittoria Cianci, Anna Mammì, Giovanbattista Gaspare Tripodi, Caterina Prestandrea, Sara Gasparini, Edoardo Ferlazzo   +8 more
wiley   +1 more source

Infection control in the brain and the eye

Acta Ophthalmologica, EarlyView.
Abstract The Central Nervous System (CNS), comprising the brain and the eye, is considered to have a ‘privileged’ mechanism for dealing with immunological challenge (immune privilege, IP). CNS IP has been revealed through experiments using foreign protein antigens and cell and tissue alloantigens (grafts), but evidence for a role for IP in modulating ...
John V. Forrester, Paul G. McMenamin, Samantha J. Dando   +2 more
wiley   +1 more source

Red blood cell membrane proteome as a reporter of disease severity, transfusion impact and genetic background in transfusion‐dependent β‐thalassaemia

British Journal of Haematology, EarlyView.
Summary Omics technologies have transformed research in haemoglobinopathies, yet the proteome of RBCs remains largely unexplored in transfusion‐dependent thalassaemia (TDT). In this proteomic analysis, Red blood cell (RBC) membranes from 48 adults with TDT were compared with healthy controls.
Konstantina Theocharaki, Sophia Delicou, Aikaterini Ntzoura‐Kani, Nikolaos Simantiris, Marianna Politou, Veroniki Komninaka, Martina Samiotaki, Jerome Zoidakis, Marianna H. Antonelou   +8 more
wiley   +1 more source

Rapid generation of prion disease models using AAV‐delivered PrP variants in knockout mice

Brain Pathology, EarlyView.
We developed a rapid AAV‐based system to generate prion disease models in weeks rather than months. Following systemic AAV9P31 delivery of modified PrP to knockout mice, we achieved brain‐wide expression and successful propagation of both classical (RML) and atypical (GSS‐A117V) prion strains.
Maitena San‐Juan‐Ansoleaga, Eva Fernández‐Muñoz, Jorge M. Charco, Enric Vidal, Diego Herrero‐Martínez, Josu Galarza‐Ahumada, Cristina Sampedro‐Torres‐Quevedo, Samanta Giler, Mariví Geijo, Gloria González‐Aseguinolaza, Hasier Eraña, Joaquín Castilla   +11 more
wiley   +1 more source

Targeting of the prion protein to the cytosol: mechanisms and consequences

, 2010
Prion diseases are characterized by the conformational transition of the cellular prion protein (PrPC) into an aberrant protein conformer, designated scrapie-prion protein (PrPSc).
Miesbauer, M., Rambold, A., Tatzelt, J., Winkelhofer, K.   +3 more
core