Results 1 to 10 of about 97,377 (307)

Treatment of Prion Disease with Heterologous Prion Proteins. [PDF]

open access: yesPLoS ONE, 2015
Prion diseases such as Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy in cattle, and scrapie in sheep are fatal neurodegenerative diseases for which there is no effective treatment.
Pamela J Skinner   +9 more
doaj   +4 more sources

Calcium-dependent serine-threonine phosphatase and autophagy inactivation mediated by Baicalein attenuates prion protein-mediated neuronal cell damage [PDF]

open access: yesBMC Complementary Medicine and Therapies
Background Prion diseases are a group of incurable and fatal neurodegenerative disorders characterized by neuronal cell death. Calcineurin and autophagy induce neurotoxicity by prion proteins, which may have therapeutic benefits by inhibiting calcineurin
Jeong-Min Hong   +5 more
doaj   +2 more sources

Genetic aspects of human prion diseases

open access: yesFrontiers in Neurology, 2022
Human prion diseases are rapidly progressive and fatal neurodegenerative conditions caused by a disease-causing isoform of the native prion protein. The prion protein gene (PRNP) encodes for the cellular prion protein, which is the biological substrate ...
Brian S. Appleby   +4 more
doaj   +1 more source

Oral administration of repurposed drug targeting Cyp46A1 increases survival times of prion infected mice

open access: yesActa Neuropathologica Communications, 2021
Prion diseases are fatal, infectious, and incurable neurodegenerative disorders caused by misfolding of the cellular prion protein (PrPC) into the infectious isoform (PrPSc). In humans, there are sporadic, genetic and infectious etiologies, with sporadic
Tahir Ali   +9 more
doaj   +1 more source

Anti-Prion Systems in Saccharomyces cerevisiae Turn an Avalanche of Prions into a Flurry

open access: yesViruses, 2022
Prions are infectious proteins, mostly having a self-propagating amyloid (filamentous protein polymer) structure consisting of an abnormal form of a normally soluble protein.
Moonil Son, Reed B. Wickner
doaj   +1 more source

Allelic Interference in Prion Replication Is Modulated by the Convertibility of the Interfering PrP C and Other Host-Specific Factors

open access: yesmBio, 2021
Prion propagation can be interfered with by the expression of a second prion protein in the host. In the present study, we investigated prion propagation in a host expressing two different prion protein genes.
Juan Carlos Espinosa   +12 more
doaj   +1 more source

Early increase and late decrease of purkinje cell dendritic spine density in prion-infected organotypic mouse cerebellar cultures. [PDF]

open access: yesPLoS ONE, 2013
Prion diseases are infectious neurodegenerative diseases associated with the accumulation of protease-resistant prion protein, neuronal loss, spongiform change and astrogliosis.
Jody L Campeau   +4 more
doaj   +1 more source

DNA methylation analysis on purified neurons and glia dissects age and Alzheimer’s disease-specific changes in the human cortex

open access: yesEpigenetics & Chromatin, 2018
Background Epigenome-wide association studies (EWAS) based on human brain samples allow a deep and direct understanding of epigenetic dysregulation in Alzheimer’s disease (AD).
Gilles Gasparoni   +16 more
doaj   +1 more source

Differential Accumulation of Misfolded Prion Strains in Natural Hosts of Prion Diseases

open access: yesViruses, 2021
Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of neurodegenerative protein misfolding diseases that invariably cause death.
Zoe J. Lambert   +3 more
doaj   +1 more source

Computational Studies of the Structural Stability of Rabbit Prion Protein Compared to Human and Mouse Prion Proteins [PDF]

open access: yes, 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
core   +2 more sources

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