Prion protein biology.
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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 Assistance Foundation, as well as by gifts from the Sherman Fairchild Foundation, Keck Foundation, G. Mathers Foundation, Bernard Osher Foundation, and Centeon.About:
This article is published in Cell.The article was published on 1998-05-01 and is currently open access. It has received 932 citations till now.read more
Citations
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Free Radicals in the Physiological Control of Cell Function
TL;DR: There is growing evidence that aging involves, in addition, progressive changes in free radical-mediated regulatory processes that result in altered gene expression.
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Intrinsically Disordered Proteins in Human Diseases: Introducing the D2 Concept
TL;DR: Overall, intriguing interconnections among intrinsic disorder, cell signaling, and human diseases suggest that protein conformational diseases may result not only from protein misfolding, but also from misidentification, missignaling, and unnatural or nonnative folding.
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Eight prion strains have PrP Sc molecules with different conformations
Jiri G. Safar,Holger Wille,Vincenza Itri,Darlene Groth,Hana Serban,Marilyn Torchia,Fred E. Cohen,Stanley B. Prusiner +7 more
TL;DR: It is indicated that each of the eight prion strains has a PrP sc molecule with a unique conformation and that the variation in incubation times is related to the relative protease sensitivity of PrPSc in each strain.
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Conformational variations in an infectious protein determine prion strain differences
TL;DR: A high-efficiency protocol for infecting yeast with the [PSI+] prion using amyloids composed of a recombinant Sup35 fragment (Sup-NM) is reported, and it is established that Sup-NM adopts an infectious conformation before entering the cell, fulfilling a key prediction of the prion hypothesis.
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Neurodegenerative diseases: a decade of discoveries paves the way for therapeutic breakthroughs.
TL;DR: Understanding of mechanisms regulating protein processing and aggregation, as well as of the toxic effects of misfolded neurodegenerative disease proteins, will facilitate development of rationally designed therapies to treat and prevent these disorders.
References
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Principles that Govern the Folding of Protein Chains
TL;DR: Anfinsen as discussed by the authors provided a sketch of the rich history of research that provided the foundation for his work on protein folding and the Thermodynamic Hypothesis, and outlined potential avenues of current and future scientific exploration.
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Novel proteinaceous infectious particles cause scrapie
TL;DR: A new term "prion" is proposed to denote a small proteinaceous infectious particle which is resistant to inactivation by most procedures that modify nucleic acids.
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Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins.
Keh-Ming Pan,Michael J. Baldwin,J Nguyen,María Gasset,Ana Serban,Darlene Groth,Ingrid Mehlhorn,Ziwei Huang,Robert J. Fletterick,Fred E. Cohen +9 more
TL;DR: It is argued that the conversion of alpha-helices into beta-sheets underlies the formation of PrPSc, and it is likely that this conformational transition is a fundamental event in the propagation of prions.
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Mice devoid of PrP are resistant to scrapie
Hansruedi Büeler,Adriano Aguzzi,Andreas W. Sailer,R.-A. Greiner,P. Autenried,Michel Aguet,Charles Weissmann +6 more
TL;DR: These experiments show that PrPC, possibly at close to normal levels, is required for the usual susceptibility to scrapie and that lack of homology between incoming prions and the host's PrP genes retards disease.
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Molecular biology of prion diseases
TL;DR: Understanding prion diseases may advance investigations of other neurodegenerative disorders and of the processes by which neurons differentiate, function for decades, and then grow senescent.