Results 11 to 20 of about 78,455 (315)
Annals of Medicine, 2000 The term 'prion diseases' refers to a group of neurodegenerative disorders thought to be caused by prions, pathogenic agents with novel modes of replication and transmission. Prion diseases are characterized by long incubation periods ranging from months to years and are invariably fatal once clinical symptoms have appeared.
Matti Haltia
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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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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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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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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 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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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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Genetic Risk Factors of Creutzfeldt-Jakob Disease in the Population of Newborns in Slovakia
Pathogens, 2021 The most frequent human prion disease is Creutzfeldt–Jakob disease (CJD). It occurs as sporadic (sCJD), genetic (gCJD), iatrogenic (iCJD) form and as variant CJD.
Dana Kosorinova +4 more
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Case report of homozygous E200D mutation of PRNP in apparently sporadic Creutzfeldt-Jakob disease
BMC Neurology, 2021 Background Inherited prion diseases are rare autosomal dominant disorders associated with diverse clinical presentations. All are associated with mutation of the gene that encodes prion protein (PRNP).
Ahamad Hassan +6 more
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Animal prion diseases: A review of intraspecies transmission [PDF]
Open Veterinary Journal, 2021 Animal prion diseases are a group of neurodegenerative, transmissible, and fatal disorders that affect several animal species. The causative agent, prion, is a misfolded isoform of normal cellular prion protein, which is found in cells with higher ...
Mauro Julián Gallardo +1 more
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