Results 31 to 40 of about 71,281 (314)
Early preclinical detection of prions in the skin of prion-infected animals
Nature Communications, 2019 A definitive pre-mortem diagnosis of prion disease depends on brain biopsy for prion detection currently and no validated alternative preclinical diagnostic tests have been reported to date.
Zerui Wang +24 more
semanticscholar +1 more source
Octarepeat region flexibility impacts prion function, endoproteolysis and disease manifestation
EMBO Molecular Medicine, 2015 The cellular prion protein (PrPC) comprises a natively unstructured N‐terminal domain, including a metal‐binding octarepeat region (OR) and a linker, followed by a C‐terminal domain that misfolds to form PrPSc in Creutzfeldt‐Jakob disease.
Agnes Lau +19 more
doaj +1 more source
Heat shock factor 1 regulates lifespan as distinct from disease onset in prion disease [PDF]
, 2008 Prion diseases are fatal, transmissible, neurodegenerative diseases caused by the misfolding of the prion protein (PrP). At present, the molecular pathways underlying prion-mediated neurotoxicity are largely unknown.
Aguzzi, Adriano +8 more
core +3 more sources
Autophagy regulates exosomal release of prions in neuronal cells
Journal of Biological Chemistry, 2018 Prions are protein-based infectious agents that autocatalytically convert the cellular prion protein PrPC to its pathological isoform PrPSc.
Basant A. Abdulrahman +2 more
semanticscholar +1 more source
Proceedings of the National Academy of Sciences, 1984 Prions are unprecedented infectious pathogens that cause a group of invariably fatal neurodegenerative diseases by an entirely novel mechanism. Prion diseases may present as genetic, infectious, or sporadic disorders, all of which involve modification of the prion protein (PrP).
openaire +3 more sources
Shortest known prion protein allele in highly BSE-susceptible lemurs [PDF]
, 2000 We describe the shortest prion protein allele known to date. Surprisingly, it is found as a polymorphism exactly in a species (prosimian lemurs) which seems highly susceptible to oral infection with BSE-derived prions. The truncation of the prion protein
Gilch, S. +2 more
core +1 more source
Pathogens, 2018 PrPSc (scrapie isoform of the prion protein) prions are the infectious agent behind diseases such as Creutzfeldt–Jakob disease in humans, bovine spongiform encephalopathy in cattle, chronic wasting disease in cervids (deer, elk, moose, and reindeer), as ...
H. Wille, J. Requena
semanticscholar +1 more source
Frontiers in Neuroscience, 2015 Organotypic cerebellar slices represent a suitable model for characterizing and manipulating prion replication in complex cell environments. Organotypic slices recapitulate prion pathology and are amenable to drug testing in the absence of a blood-brain ...
Hanna eWolf +7 more
doaj +1 more source
Frontiers in Molecular Biosciences, 2023 Prion diseases are a group of infectious neurodegenerative diseases produced by the conversion of the normal prion protein (PrPC) into the disease-associated form (PrPSc).
Luis Concha-Marambio +9 more
doaj +1 more source
Mineral licks as environmental reservoirs of chronic wasting disease prions
PLoS ONE, 2018 Chronic wasting disease (CWD) is a fatal neurodegenerative disease of deer, elk, moose, and reindeer (cervids) caused by misfolded prion proteins. The disease has been reported across North America and recently discovered in northern Europe. Transmission
Ian H Plummer +4 more
semanticscholar +1 more source