Results 31 to 40 of about 97,942 (322)

Canine detection of chronic wasting disease (CWD) in laboratory and field settings

open access: yesPrion, 2023
Chronic wasting disease (CWD) is a fatal transmissible spongiform encephalopathy that affects both free-ranging and farmed cervid species, including mule deer, white-tailed deer, and elk (Odocoileus hemionus, Odocoileus virginianus, and Cervus canadensis)
Amritha Mallikarjun   +11 more
doaj   +1 more source

Prions [PDF]

open access: yesProceedings 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

Serial changes in regional cerebral blood flow in Gerstmann–Sträussler–Scheinker disease caused by a Pro-to-Leu mutation at codon 105 in the prion protein gene

open access: yesPrion, 2023
Gerstmann–Sträussler–Scheinker disease with a Pro-to-Leu substitution at codon 105 in the prion protein gene (GSS-P105L) is a rare variant of human genetic prion disease.
Honami Kawai   +3 more
doaj   +1 more source

Expression of the cellular prion protein by mast cells in the human carotid body

open access: yesPrion, 2023
Prion diseases are fatal neurologic disorders that can be transmitted by blood transfusion. The route for neuroinvasion following exposure to infected blood is not known.
Gregory D. Sweetland   +4 more
doaj   +1 more source

Estimating sequence diversity of prion protein gene (PRNP) in Portuguese populations of two cervid species: red deer and fallow deer

open access: yesPrion, 2023
Among the transmissible spongiform encephalopathies (TSEs), chronic wasting disease (CWD) in cervids is now a rising concern in wildlife within Europe, after the detection of the first case in Norway in 2016, in a wild reindeer and until June 2022 a ...
Jorge C. Pereira   +21 more
doaj   +1 more source

Prion-like domain mutations in hnRNPs cause multisystem proteinopathy and ALS

open access: yesNature, 2013
Summary Algorithms designed to identify canonical yeast prions predict that ~250 human proteins, including several RNA-binding proteins associated with neurodegenerative disease, harbor a distinctive prion-like domain (PrLD) enriched in uncharged polar ...
Hong-Joo Kim   +36 more
semanticscholar   +1 more source

Variability in prion protein genotypes by spatial unit to inform susceptibility to chronic wasting disease

open access: yesPrion, 2022
Chronic wasting disease (CWD) is a fatal encephalopathy affecting North American cervids. Certain alleles in a host’s prion protein gene are responsible for reduced susceptibility to CWD.
Alberto F. Fameli   +4 more
doaj   +1 more source

Cellular Prion Protein Mediates Impairment of Synaptic Plasticity by Amyloid-β Oligomers

open access: yesNature, 2009
A pathological hallmark of Alzheimer’s disease is an accumulation of insoluble plaque containing the amyloid-β peptide of 40–42 amino acid residues. Prefibrillar, soluble oligomers of amyloid-β have been recognized to be early and key intermediates in ...
J. Laurén   +4 more
semanticscholar   +1 more source

What is the evidence that tau pathology spreads through prion-like propagation?

open access: yesActa Neuropathologica Communications, 2017
Emerging experimental evidence suggests that the spread of tau pathology in the brain in Tauopathies reflects the propagation of abnormal tau species along neuroanatomically connected brain areas.
A. Mudher   +10 more
semanticscholar   +1 more source

Prion-Like Propagation of Protein Misfolding and Aggregation in Amyotrophic Lateral Sclerosis

open access: yesFrontiers in Molecular Neuroscience, 2019
The discovery that prion protein can misfold into a pathological conformation that encodes structural information capable of both propagation and inducing severe neuropathology has revolutionized our understanding of neurodegenerative disease.
Luke McAlary   +3 more
semanticscholar   +1 more source

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