Transmissible Proteins: Expanding the Prion Heresy
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چکیده
منابع مشابه
Transmissible Proteins: Expanding the Prion Heresy
The once heretical concept that a misfolded protein is the infectious agent responsible for prion diseases is now widely accepted. Recent exciting research has led not only to the end of the skepticism that proteins can transmit disease, but also to expanding the concept that transmissible proteins might be at the root of some of the most prevalent human illnesses. At the same time, the idea th...
متن کاملExpanding the prion disease repertoire.
In 1982, Stanley Prusiner proposed that the infectious agent of transmissible spongiform encephalopathies (TSEs), a group of relatively rare neurodegenerative disorders that includes Creutzfeldt–Jakob disease and scrapie, lacks replicating nucleic acids and instead is composed primarily of a misfolded conformation of the prion protein, which he termed PrP (1). Originally, the “prion” hypothesis...
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The transmissible agent of prion disease consists of prion protein (PrP) in β-sheet-rich state (PrP(Sc)) that can replicate its conformation according to a template-assisted mechanism. This mechanism postulates that the folding pattern of a newly recruited polypeptide accurately reproduces that of the PrP(Sc) template. Here, three conformationally distinct amyloid states were prepared in vitro ...
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Prion diseases, also called transmissible spongiform encephalopathies (TSEs), lead to neurological dysfunction in animals and are fatal. Infectious prion proteins are causative agents of many mammalian TSEs, including scrapie (in sheep), chronic wasting disease (in deer and elk), bovine spongiform encephalopathy (BSE; in cattle), and Creutzfeldt-Jakob disease (CJD; in humans). BSE, better known...
متن کاملAstrocytes in transmissible spongiform encephalopathies (prion diseases).
Astrocytosis is one of the hallmarks of neuropathology of transmissible spongiform encephalopathies (TSEs) or prion diseases. In this review, we summarize data on astrocytic reaction, including naturally occurring or experimentally induced TSEs. A particular form of astrocytic reaction is known as gliocytosis and it is typical for experimental scrapie in mouse or in hamsters. Also, astrocytes p...
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ژورنال
عنوان ژورنال: Cell
سال: 2012
ISSN: 0092-8674
DOI: 10.1016/j.cell.2012.05.007