Results 1 to 10 of about 97,942 (322)
2.7 Å cryo-EM structure of ex vivo RML prion fibrils
Nature Communications, 2022 High-resolution structures of mammalian prions have remained elusive. Here, Manka et al. report the cryo-EM structure of infectious RML prion fibrils from mice.
Szymon W. Manka +7 more
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Prion-Prion Interactions [PDF]
Prion, 2007 The term prion has been used to describe self-replicating protein conformations that can convert other protein molecules of the same primary structure into its prion conformation. Several different proteins have now been found to exist as prions in Saccharomyces cerevisiae. Surprisingly, these heterologous prion proteins have a strong influence on each
Irina L, Derkatch, Susan W, Liebman
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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 +5 more
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Scientific Reports, 2021 The cerebrospinal fluid (CSF) real-time quaking-induced conversion assay (RT-QuIC) is an ultrasensitive prion amyloid seeding assay for diagnosis of sporadic Creutzfeldt–Jakob disease (CJD) but several prion strains remain unexplored or resistant to ...
Tze How Mok +7 more
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Prion protein gene mutation detection using long-read Nanopore sequencing
Scientific Reports, 2022 Prion diseases are fatal neurodegenerative conditions that affect humans and animals. Rapid and accurate sequencing of the prion gene PRNP is paramount to human prion disease diagnosis and for animal surveillance programmes.
François Kroll +6 more
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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 +7 more
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Cold Spring Harbor Protocols, 2017 Infectious proteins (prions) are usually self-templating filamentous protein polymers (amyloids). Yeast prions are genes composed of protein and, like the multiple alleles of DNA-based genes, can have an array of “variants,” each a distinct self-propagating amyloid conformation. Like the lethal mammalian prions and amyloid diseases, yeast prions may be
Dmitry, Kryndushkin +3 more
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Essential Components of Synthetic Infectious Prion Formation De Novo
Biomolecules, 2022 Prion diseases are a class of neurodegenerative diseases that are uniquely infectious. Whilst their general replication mechanism is well understood, the components required for the formation and propagation of highly infectious prions are poorly ...
Kezia Jack +2 more
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Arquivos de Neuro-Psiquiatria, 1991 Os autores se propõem a revisar alguns aspectos básicos sobre os prions, alertando sobre a possível participação destes na etiologia de algumas enfermidades degenerativas do sistema nervoso.
Godoy, J. M. +2 more
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Prion protein conversion at two distinct cellular sites precedes fibrillisation
Nature Communications, 2023 The self-templating nature of prions plays a central role in prion pathogenesis and is associated with infectivity and transmissibility. Since propagation of proteopathic seeds has now been acknowledged a principal pathogenic process in many types of ...
Juan Manuel Ribes +5 more
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