The prion strain phenomenon: Molecular basis and unprecedented features
نویسندگان
چکیده
منابع مشابه
Molecular basis of prion diseases.
Fatal neurodegenerative diseases have been known for more than 300 years. As early as 1759, the scrapie disease of sheep was mentioned as a lethal and transmissible disease in a german veterinary textbook by Leopoldt (1759). Experimental transmission was shown in 1936 by Cuillé (1938). Not knowing about the similarity to the sheep disease, the neurologists Alfons Jakob (1921) and Hans Creutzfel...
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We consider a model for the polymerization (fragmentation) process involved in infectious prion self-replication and study both its dynamics and non-zero steady state. We address several issues. Firstly, we give conditions leading to size repartitions of PrPsc aggregates that exhibit bimodal distributions, as indicated by recent experimental studies of prion aggregates distribution [19]. This i...
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The most seductive questions in prion biology have always centered around the unusual nature of disease transmissibility1,2. What is the transmissible agent? What are the routes of transmission from one organism to another? How does the transmissible agent spread within an organism? What is the mechanism of replication? What is the nature of the barrier to transmission between species? Although...
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Prions of budding yeast serve as a tractable model of amyloid behavior. Here we address the issue of the effect of yeast strain variation on prion stability, focusing also on the effect of amyloid conformation and the involvement of the co-chaperone Sis1, an essential J-protein partner of Hsp70. We found, despite an initial report to the contrary, that yeast strain background has little effect ...
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The yeast [PSI+] factor is inherited by a prion mechanism involving self-propagating Sup35p aggregates. We find that Sup35p prion function is conserved among distantly related yeasts. As with mammalian prions, a species barrier inhibits prion induction between Sup35p from different yeast species. This barrier is faithfully reproduced in vitro where, remarkably, ongoing polymerization of one Sup...
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ژورنال
عنوان ژورنال: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
سال: 2007
ISSN: 0925-4439
DOI: 10.1016/j.bbadis.2006.12.006