Experimental models of prion‐like protein propagation
Prion‐like propagation has been proposed to underlie the pathogenesis and progression of many progressive neurodegenerative diseases, and considerable experimental evidence has been accumulated to support this idea. However, only limited evidence is available from the brains of patients, and it is not clear how well various experimental models reflect ...
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Prion-Like Domains in Phagobiota
Prions are molecules characterized by self-propagation, which can undergo a conformational switch leading to the creation of new prions. Prion proteins have originally been associated with the development of mammalian pathologies; however, recently they ...
George Tetz, Victor Tetz
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Spreading of a prion domain from cell-to-cell by vesicular transport in Caenorhabditis elegans. [PDF]
Prion proteins can adopt self-propagating alternative conformations that account for the infectious nature of transmissible spongiform encephalopathies (TSEs) and the epigenetic inheritance of certain traits in yeast.
Carmen I Nussbaum-Krammer +4 more
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Evidence that corticofugal propagation of ALS pathology is not mediated by prion-like mechanism
Amyotrophic lateral sclerosis (ALS) arises from the combined degeneration of motor neurons (MN) and corticospinal neurons (CSN). Recent clinical and pathological studies suggest that ALS might start in the motor cortex and spread along the corticofugal axonal projections (including the CSN), either via altered cortical excitability and activity or via ...
Scekic-Zahirovic, Jelena +9 more
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The role of Hsp70 chaperones in yeast prion propagation is well established. Highly conserved Hsp90 chaperones participate in a number of cellular processes, such as client protein maturation, protein degradation, cellular signalling and apoptosis, but ...
Navinder Kumar +4 more
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Conformational propagation with prion‐like characteristics in a simple model of protein folding [PDF]
AbstractProtein refolding/misfolding to an alternative form plays an aetiologic role in many diseases in humans, including Alzheimer's disease, the systemic amyloidoses, and the prion diseases. Here we have discovered that such refolding can occur readily for a simple lattice model of proteins in a propagatable manner without designing for any ...
P M, Harrison +3 more
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Detection of classical BSE prions in asymptomatic cows after inoculation with atypical/Nor98 scrapie
The emergence of bovine spongiform encephalopathy (BSE) prions from atypical scrapie has been recently observed upon experimental transmission to rodent and swine models. This study aimed to assess whether the inoculation of atypical scrapie could induce
Marina Betancor +11 more
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Prion protein self-peptides modulate prion interactions and conversion [PDF]
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. +12 more
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Proteinase K-Resistant Material in ARR/VRQ Sheep Brain Affected with Classical Scrapie Is Composed Mainly of VRQ Prion Protein [PDF]
Classical scrapie is a prion disease in sheep and goats. In sheep, susceptibility to disease is genetically influenced by single amino acid substitutions.
Bossers, A. +28 more
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The prion-like transmission of tau oligomers via exosomes
The conversion and transmission of misfolded proteins established the basis for the prion concept. Neurodegenerative diseases are considered “prion-like” disorders that lack infectivity.
Noel A. Jackson +2 more
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