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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The Future of Seed Amplification Assays and Clinical Trials
Frontiers in Aging Neuroscience, 2022 Prion-like seeded misfolding of host proteins is the leading hypothesised cause of neurodegenerative diseases. The exploitation of the mechanism in the protein misfolding cyclic amplification (PMCA) and real-time quaking-induced conversion (RT-QuIC ...
Thomas Coysh +3 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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Simultaneous expression of MMB-FOXM1 complex components enables efficient bypass of senescence
Scientific Reports, 2021 Cellular senescence is a stable cell cycle arrest that normal cells undergo after a finite number of divisions, in response to a variety of intrinsic and extrinsic stimuli. Although senescence is largely established and maintained by the p53/p21WAF1/CIP1
Ruchi Kumari +7 more
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PRION DISEASES. PATHOGENS OF PRION DISEASES
Актуальные проблемы теоретической и клинической медицины, 2023 Prion diseases are diseases associated with pathological proteins and based on a protein with an abnormal tertiary structure. Prions are a special class of infectious pathogens that do not have nucleic acids in their composition.
G. Seribekkyzy, V. Galimullin
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Computational Studies of the Structural Stability of Rabbit Prion Protein Compared to Human and Mouse Prion Proteins [PDF]
, 2011 Prion diseases are invariably fatal and highly infectious neurodegenerative diseases affecting humans and animals. The neurodegenerative diseases such as Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob diseases, Gerstmann-Str$\ddot{a}$ussler ...
Zhang, Jiapu
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