Results 11 to 20 of about 50,704 (271)

Overexpression of mouse prion protein in transgenic mice causes a non-transmissible spongiform encephalopathy

Scientific Reports, 2022
Transgenic mice over-expressing human PRNP or murine Prnp transgenes on a mouse prion protein knockout background have made key contributions to the understanding of human prion diseases and have provided the basis for many of the fundamental advances in
Graham S. Jackson, Jacqueline Linehan, Sebastian Brandner, Emmanuel A. Asante, Jonathan D. F. Wadsworth, John Collinge   +5 more
doaj   +1 more source

DNA methylation analysis on purified neurons and glia dissects age and Alzheimer’s disease-specific changes in the human cortex

Epigenetics & Chromatin, 2018
Background Epigenome-wide association studies (EWAS) based on human brain samples allow a deep and direct understanding of epigenetic dysregulation in Alzheimer’s disease (AD).
Gilles Gasparoni, Sebastian Bultmann, Pavlo Lutsik, Theo F. J. Kraus, Sabrina Sordon, Julia Vlcek, Vanessa Dietinger, Martina Steinmaurer, Melanie Haider, Christopher B. Mulholland, Thomas Arzberger, Sigrun Roeber, Matthias Riemenschneider, Hans A. Kretzschmar, Armin Giese, Heinrich Leonhardt, Jörn Walter   +16 more
doaj   +1 more source

Prions and Prion-like Proteins [PDF]

Journal of Biological Chemistry, 2014
Prions are self-replicating protein aggregates and are the primary causative factor in a number of neurological diseases in mammals. The prion protein (PrP) undergoes a conformational transformation leading to aggregation into an infectious cellular pathogen.
openaire   +2 more sources

Early increase and late decrease of purkinje cell dendritic spine density in prion-infected organotypic mouse cerebellar cultures. [PDF]

PLoS ONE, 2013
Prion diseases are infectious neurodegenerative diseases associated with the accumulation of protease-resistant prion protein, neuronal loss, spongiform change and astrogliosis.
Jody L Campeau, Gengshu Wu, John R Bell, Jay Rasmussen, Valerie L Sim   +4 more
doaj   +1 more source

ALK1 controls hepatic vessel formation, angiodiversity, and angiocrine functions in hereditary hemorrhagic telangiectasia of the liver

Hepatology, EarlyView., 2022
Hepatic endothelial Alk1 signaling protects from development of vascular malformations while maintaining organ‐specific endothelial differentiation and angiocrine portmanteau of the names Wingless and Int‐1 signaling. Abstract Background and Aims In hereditary hemorrhagic telangiectasia (HHT), severe liver vascular malformations are associated with ...
Christian David Schmid, Victor Olsavszky, Manuel Reinhart, Vanessa Weyer, Felix A. Trogisch, Carsten Sticht, Manuel Winkler, Sina W. Kürschner, Johannes Hoffmann, Roxana Ola, Theresa Staniczek, Joerg Heineke, Beate K. Straub, Jens Mittler, Kai Schledzewski, Peter ten Dijke, Karsten Richter, Steven Dooley, Cyrill Géraud, Sergij Goerdt, Philipp‐Sebastian Koch   +20 more
wiley   +1 more source

Membrane-enriched proteome changes and prion protein expression during neural differentiation and in neuroblastoma cells

BMC Genomics, 2017
Background The function of the prion protein, involved in the so-called prion diseases, remains a subject of intense debate and the possibility that it works as a pleiotropic protein through the interaction with multiple membrane proteins is somehow ...
J. A. Macedo, D. Schrama, I. Duarte, E. Tavares, J. Renaut, M. E. Futschik, P. M. Rodrigues, E. P. Melo   +7 more
doaj   +1 more source

Intraperitoneal Infection of Wild-Type Mice with Synthetically Generated Mammalian Prion. [PDF]

PLoS Pathogens, 2015
The prion hypothesis postulates that the infectious agent in transmissible spongiform encephalopathies (TSEs) is an unorthodox protein conformation based agent.
Xinhe Wang, Gillian McGovern, Yi Zhang, Fei Wang, Liang Zha, Martin Jeffrey, Jiyan Ma   +6 more
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Novel insertion/deletion polymorphisms and genetic features of the shadow of prion protein gene (SPRN) in dogs, a prion-resistant animal

Frontiers in Veterinary Science, 2022
Prion diseases are fatal infectious neurodegenerative disorders that are induced by misfolded prion protein (PrPSc). Previous studies have reported that the shadow of prion protein (Sho) encoded by the shadow of prion protein gene (SPRN) plays a critical
Yong-Chan Kim, Yong-Chan Kim, Hyeon-Ho Kim, Hyeon-Ho Kim, An-Dang Kim, Byung-Hoon Jeong, Byung-Hoon Jeong   +6 more
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Differential Accumulation of Misfolded Prion Strains in Natural Hosts of Prion Diseases

Viruses, 2021
Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of neurodegenerative protein misfolding diseases that invariably cause death.
Zoe J. Lambert, Justin J. Greenlee, Eric D. Cassmann, M. Heather West Greenlee   +3 more
doaj   +1 more source

Deposition pattern and subcellular distribution of disease-associated prion protein in cerebellar organotypic slice cultures infected with scrapie

Frontiers in Neuroscience, 2015
Organotypic cerebellar slices represent a suitable model for characterizing and manipulating prion replication in complex cell environments. Organotypic slices recapitulate prion pathology and are amenable to drug testing in the absence of a blood-brain ...
Hanna eWolf, Andre eHossinger, Andrea eFehlinger, Sven eBuettner, Valerie eSim, Debbie eMcKenzie, Ina Maja Vorberg, Ina Maja Vorberg   +7 more
doaj   +1 more source