Results 1 to 10 of about 50,704 (271)
Treatment of Prion Disease with Heterologous Prion Proteins. [PDF]
PLoS 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
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Calcium-dependent serine-threonine phosphatase and autophagy inactivation mediated by Baicalein attenuates prion protein-mediated neuronal cell damage [PDF]
BMC Complementary Medicine and TherapiesBackground 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
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Cellular prion protein and calcium ions trigger the neurotoxicity of α-synuclein aggregates. [PDF]
Cell BiosciBigi A +7 more
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Genetic aspects of human prion diseases
Frontiers 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
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Acta 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
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Anti-Prion Systems in Saccharomyces cerevisiae Turn an Avalanche of Prions into a Flurry
Viruses, 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
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mBio, 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
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Prion protein glycosylation [PDF]
Journal of Neurochemistry, 2005 AbstractThe transmissible spongiform encephalopathies (TSE), or prion diseases are a group of transmissible neurodegenerative disorders of humans and animals. Although the infectious agent (the ‘prion’) has not yet been formally defined at the molecular level, much evidence exists to suggest that the major or sole component is an abnormal isoform of ...
Lawson, Victoria A +3 more
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Prion Protein Misfolding [PDF]
Current Molecular Medicine, 2009 The crucial event in the development of transmissible spongiform encephalopathies (TSEs) is the conformational change of a host-encoded membrane protein - the cellular PrP(C) - into a disease associated, fibril-forming isoform PrP(Sc). This conformational transition from the alpha-helix-rich cellular form into the mainly beta-sheet containing ...
Kupfer, L, Hinrichs, W, Groschup, M.H
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Cell, 1998 We thank G. Carlson, N. Nathanson, and J. Safar for carefully reviewing sections of this manuscript. This research was supported by grants from the National Institute of Aging and the National Institute of Neurologic Diseases and Stroke of the National Institutes of Health, International Human Frontiers of Science Program, and American Health ...
Prusiner, Stanley B +3 more
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