Results 11 to 20 of about 4,254,953 (206)

The Quest for Cellular Prion Protein Functions in the Aged and Neurodegenerating Brain

Cells, 2020
Cellular (also termed ‘natural’) prion protein has been extensively studied for many years for its pathogenic role in prionopathies after misfolding.
Rosalina Gavín, Laia Lidón, Isidre Ferrer, José Antonio del Río   +3 more
doaj   +1 more source

Unexpected decrease of full-length prion protein in macaques inoculated with prion-contaminated blood products

Frontiers in Molecular Biosciences, 2023
The presence of prion infectivity in the blood of patients affected by variant Creutzfeldt–Jakob disease (v-CJD), the human prion disease linked to the bovine spongiform encephalopathy (BSE), poses the risk of inter-human transmission of this fatal prion
Nina Jaffré, Jérôme Delmotte, Jacqueline Mikol, Jean-Philippe Deslys, Emmanuel Comoy   +4 more
doaj   +1 more source

Prion protein self-peptides modulate prion interactions and conversion [PDF]

, 2009
Background: Molecular mechanisms underlying prion agent replication, converting host-encoded cellular prion protein (PrPC) into the scrapie associated isoform (PrPSc), are poorly understood.
Bossers, A., Fred G van Zijderveld, Priem, J., Drophatie Timmers-Parohi, Rigter, A., Alex Bossers, Alan Rigter, Timmers-Parohi, D., Jan PM Langeveld, van Zijderveld, F.G., Zijderveld, F.G., van, Jan Priem, Langeveld, J.P.M.   +12 more
core   +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

Transport of Prions in the Peripheral Nervous System: Pathways, Cell Types, and Mechanisms

Viruses, 2022
Prion diseases are transmissible protein misfolding disorders that occur in animals and humans where the endogenous prion protein, PrPC, undergoes a conformational change into self-templating aggregates termed PrPSc.
Sam M. Koshy, Anthony E. Kincaid, Jason C. Bartz   +2 more
doaj   +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

Enhanced phosphorylation of PERK in primary cultured neurons as an autonomous neuronal response to prion infection.

PLoS ONE, 2020
Conversion of cellular prion protein (PrPC) into the pathogenic isoform of prion protein (PrPSc) in neurons is one of the key pathophysiological events in prion diseases.
Misaki Tanaka, Takeshi Yamasaki, Rie Hasebe, Akio Suzuki, Motohiro Horiuchi   +4 more
doaj   +1 more source

Effects of cellular prion protein on rapid eye movement sleep deprivation-induced spatial memory impairment

Journal of Integrative Neuroscience, 2019
The effects of cellular prion protein on rapid eye movement sleep deprivation-induced spatial memory impairment were investigated, and the related mechanisms explored.
Li Hu, Peng Li, Zhendong You, Zhaohuan Zhang, Hongyang Jin, Liuqing Huang   +5 more
doaj   +1 more source

Transcriptomic analysis identifies novel potential biomarkers and highlights cilium-related biological processes in the early stages of prion disease in mice

Prion, 2022
Prion diseases are fatal and irreversible neurodegenerative diseases induced by the pathogenic form of the prion protein (PrPSc), which is converted from the benign form of the prion protein (PrPC).
Yong-Chan Kim, Byung-Hoon Jeong
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