Neurobiology of Disease A New Mechanism for Transmissible Prion Diseases
نویسندگان
چکیده
Natallia Makarava,1 Gabor G. Kovacs,2 Regina Savtchenko,1 Irina Alexeeva,3 Valeriy G. Ostapchenko,1 Herbert Budka,2 Robert G. Rohwer,3 and Ilia V. Baskakov1,4 1Center for Biomedical Engineering and Technology, University of Maryland, Baltimore, Maryland 21201, 2Institute of Neurology, Medical University of Vienna, A-1097 Vienna, Austria, 3Medical Research Service, Veterans Affairs Medical Center, University of Maryland, Baltimore, Maryland 21201, and 4Department of Anatomy and Neurobiology, University of Maryland School of Medicine, Baltimore, Maryland 21201
منابع مشابه
Genesis of Mammalian Prions: From Non-infectious Amyloid Fibrils to a Transmissible Prion Disease
The transmissible agent of prion disease consists of a prion protein in its abnormal, β-sheet rich state (PrP(Sc)), which is capable of replicating itself according to the template-assisted mechanism. This mechanism postulates that the folding pattern of a newly recruited polypeptide chain accurately reproduces that of a PrP(Sc) template. Here we report that authentic PrP(Sc) and transmissible ...
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The human transmissible spongiform encephalopathies or human prion diseases are one of the most intensively investigated groups of rare human neurodegenerative conditions. They are generally held to be unique in terms of their complex epidemiology and phenotypic variability, but they may also serve as a paradigm with which other more common protein misfolding disorders might be compared and con...
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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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The infectious cause of the transmissible spongiform encephalopathies (TSEs), or prion diseases, is not yet clearly defined. Although minorities of researchers cling tenaciously to the virus hypothesis, the prion or protein-only hypothesis is now widely accepted by most scientists working in this field (1). When originally formulated, the protein-only hypothesis proposed that the TSE agent was ...
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Prion replication occurs via a template-assisted mechanism, which postulates that the folding pattern of a newly recruited polypeptide chain accurately reproduces that of a template. The concept of prion-like template-assisted propagation of an abnormal protein conformation has been expanded to amyloidogenic proteins associated with Alzheimer, Parkinson, Huntington diseases, amyotrophic lateral...
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