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Prion-like disorders and Transmissible Spongiform Encephalopathies: An overview of the mechanistic features that are shared by the various disease-related misfolded proteins

Eraña, Hasier; Venegas, Vanesa; Moreno, Jorge; Castilla, Joaquín


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    "description": "Prion diseases or Transmissible Spongiform Encephalopathies\n(TSEs) are a group of fatal neurodegenerative disorders affecting several\nmammalian species. Its causative agent, disease-associated prion protein\n(PrPd), is a self-propagating \u03b2-sheet rich aberrant conformation of the\ncellular prion protein (PrPC) with neurotoxic and aggregation-prone\nproperties, capable of inducing misfolding of PrPC molecules. PrPd is the\nmajor constituent of prions and, most importantly, is the first known\nexample of a protein with infectious attributes. It has been suggested\nthat similar molecular mechanisms could be shared by other proteins\nimplicated in diseases such as Alzheimer's disease, Parkinson's disease,\namyotrophic lateral sclerosis or systemic amyloidoses. Accordingly,\nseveral terms have been proposed to collectively group all these\ndisorders. Through the stringent evaluation of those aspects that\ncharacterise TSE-causing prions, in particular propagation and spread,\nstrain variability or transmissibility, we will discuss whether terms\nsuch as \"prion\", \"prion-like\", \"prionoid\" or \"propagon\" can be used when\nreferring to the aetiological agents of the above other disorders.\nMoreover, it will also be discussed whether the term \"infectious\", which\ndefines a prion essential trait, is currently misused when referring to\nthe other misfolded proteins.", 
    "license": {
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    "title": "Prion-like disorders and Transmissible Spongiform Encephalopathies: An overview of the mechanistic features that are shared by the various disease-related misfolded proteins", 
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    "publication_date": "2017-02-01", 
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        "name": "Era\u00f1a, Hasier"
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        "name": "Venegas, Vanesa"
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