Results 61 to 70 of about 5,604,422 (355)

Prion and Prion-Like Protein Strains: Deciphering the Molecular Basis of Heterogeneity in Neurodegeneration

Viruses, 2019
Increasing evidence suggests that neurodegenerative disorders share a common pathogenic feature: the presence of deposits of misfolded proteins with altered physicochemical properties in the Central Nervous System.
C. Scialò, E. De Cecco, P. Manganotti, G. Legname   +3 more
semanticscholar   +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

Thermodynamic Stabilization of the Folded Domain of Prion Protein Inhibits Prion Infection in Vivo

Cell Reports, 2013
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are associated with the conformational conversion of the cellular prion protein, PrPC, into a protease-resistant form, PrPSc.
Qingzhong Kong, Jeffrey L. Mills, Bishwajit Kundu, Xinyi Li, Liuting Qing, Krystyna Surewicz, Ignazio Cali, Shenghai Huang, Mengjie Zheng, Wieslaw Swietnicki, Frank D. Sönnichsen, Pierluigi Gambetti, Witold K. Surewicz   +12 more
doaj   +1 more source

A high-content neuron imaging assay demonstrates inhibition of prion disease-associated neurotoxicity by an anti-prion protein antibody

Scientific Reports, 2022
There is an urgent need to develop disease-modifying therapies to treat neurodegenerative diseases which pose increasing challenges to global healthcare systems.
Madeleine Reilly, Iryna Benilova, Azadeh Khalili-Shirazi, Christian Schmidt, Parvin Ahmed, Daniel Yip, Parmjit S. Jat, John Collinge   +7 more
doaj   +1 more source

A naturally occurring variant of the human prion protein completely prevents prion disease

Nature, 2015
Mammalian prions, transmissible agents causing lethal neurodegenerative diseases, are composed of assemblies of misfolded cellular prion protein (PrP). A novel PrP variant, G127V, was under positive evolutionary selection during the epidemic of kuru—an ...
E. Asante, Michelle Smidak, Andrew Grimshaw, Richard Houghton, Andrew Tomlinson, Asif Jeelani, T. Jakubcova, Shyma Hamdan, A. Richard-Londt, J. Linehan, S. Brandner, M. Alpers, J. Whitfield, S. Mead, J. Wadsworth, J. Collinge   +15 more
semanticscholar   +1 more source

Cytosolic Prion Protein Toxicity Is Independent of Cellular Prion Protein Expression and Prion Propagation [PDF]

Journal of Virology, 2007
ABSTRACT Prion diseases are transmissible neurodegenerative diseases caused by a conformational isoform of the prion protein (PrP), a host-encoded cell surface sialoglycoprotein. Recent evidence suggests a cytosolic fraction of PrP (cyPrP) functions either as an initiating factor or toxic element of prion disease.
James A. Mastrianni, Mark F. Ciaccio, Eric M. Norstrom, Benjamin Rassbach, Robert L. Wollmann   +4 more
openaire   +2 more sources

Intra- and interspecies interactions between prion proteins and effects of mutations and polymorphisms [PDF]

, 2003
Recently, crystallization of the prion protein in a dimeric form was reported. Here we show that native soluble homogenous FLAG-tagged prion proteins from hamster, man and cattle expressed in the baculovirus system are predominantly dimeric.
Bradley R., Campbell T.A., Clark W.W., Cutlip R.C., Edenhofer F., Goldfarb L.G., Krasemann S., Leucht C., Lopez L., Majtenyi C., Scott M.R., Weiss S.   +11 more
core   +1 more source

Bank Vole Prion Protein As an Apparently Universal Substrate for RT-QuIC-Based Detection and Discrimination of Prion Strains

PLoS Pathogens, 2015
Prions propagate as multiple strains in a wide variety of mammalian species. The detection of all such strains by a single ultrasensitive assay such as Real Time Quaking-induced Conversion (RT-QuIC) would facilitate prion disease diagnosis, surveillance ...
C. Orrú, B. Groveman, L. Raymond, A. Hughson, R. Nonno, W. Zou, B. Ghetti, P. Gambetti, B. Caughey   +8 more
semanticscholar   +1 more source

Functions of the Prion Protein

, 2017
Although initially disregarded compared to prion pathogenesis, the functions exerted by the cellular prion protein PrPC have gained much interest over the past two decades. Research aiming at unraveling PrPC functions started to intensify when it became appreciated that it would give clues as to how it is subverted in the context of prion infection and,
Théo Z. Hirsch, Séverine Martin-Lannerée, Sophie Mouillet-Richard, Sophie Mouillet-Richard   +3 more
openaire   +3 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). PrPC features a globular C-terminal domain containing 3 α-helices and a short β-sheet and a long flexible N-terminal tail whose exact conformation in vivo is not yet known and a metastable subdomain
openaire   +5 more sources