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Science 14 June 1991: Vol. 252. no. 5012, pp. 1515 - 1522 DOI: 10.1126/science.1675487
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Articles
Science, Vol 252, Issue 5012, 1515-1522
Copyright © 1991 by American Association for the Advancement of Science
Molecular biology of prion diseases
SB Prusiner
Department of Neurology, University of California, San Francisco 94143.
Prions cause transmissible and genetic neurodegenerative diseases, including scrapie and bovine spongiform encephalopathy of animals and Creutzfeldt-Jakob and Gerstmann-Straussler-Scheinker diseases of humans. Infectious prion particles are composed largely, if not entirely, of an abnormal isoform of the prion protein, which is encoded by a chromosomal gene. A posttranslational process, as yet unidentified, converts the cellular prion protein into an abnormal isoform. Scrapie incubation times, neuropathology, and prion synthesis in transgenic mice are controlled by the prion protein gene. Point mutations in the prion protein genes of animals and humans are genetically linked to development of neuro-degeneration. Transgenic mice expressing mutant prion proteins spontaneously develop neurologic dysfunction and spongiform neuropathology. 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.
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- Sheep Prion Protein Synthetic Peptide Spanning Helix 1 and beta -Strand 2 (Residues 142-166) Shows beta -Hairpin Structure in Solution.
- S. A. Kozin, G. Bertho, A. K. Mazur, H. Rabesona, J.-P. Girault, T. Haertle, M. Takahashi, P. Debey, and G. H. B. Hoa (2001)
J. Biol. Chem.
276, 46364-46370
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- Prion Protein Fragment PrP-(106-126) Induces Apoptosis via Mitochondrial Disruption in Human Neuronal SH-SY5Y Cells.
- C. N. O'Donovan, D. Tobin, and T. G. Cotter (2001)
J. Biol. Chem.
276, 43516-43523
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- Deletion of {beta}-Strand and {alpha}-Helix Secondary Structure in Normal Prion Protein Inhibits Formation of Its Protease-Resistant Isoform.
- I. Vorberg, K. Chan, and S. A. Priola (2001)
J. Virol.
75, 10024-10032
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- A Critical Review of Atypical Cerebellum-Type Creutzfeldt-Jakob Disease: Its Relationship to ``New Variant'' CJD and Bovine Spongiform Encephalopathy.
- H. K. Narang (2001)
Experimental Biology and Medicine
226, 629-639
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- Neurodegenerative Diseases and Prions.
- S. B. Prusiner (2001)
N. Engl. J. Med.
344, 1516-1526
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- PoPMuSiC, an algorithm for predicting protein mutant stability changes. Application to prion proteins.
- D. Gilis and M. Rooman (2000)
Protein Eng. Des. Sel.
13, 849-856
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- Physiological Expression of the Gene for PrP-Like Protein, PrPLP/Dpl, by Brain Endothelial Cells and its Ectopic Expression in Neurons of PrP-Deficient Mice Ataxic Due to Purkinje Cell Degeneration.
- A. Li, S. Sakaguchi, K. Shigematsu, R. Atarashi, B. C. Roy, R. Nakaoke, K. Arima, N. Okimura, J. Kopacek, and S. Katamine (2000)
Am. J. Pathol.
157, 1447-1452
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- Strain-specific propagation of PrPSc properties into baculovirus-expressed hamster PrPC.
- V. Iniguez, D. McKenzie, J. Mirwald, and J. Aiken (2000)
J. Gen. Virol.
81, 2565-2571
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- Molecular dynamics simulation of human prion protein including both N-linked oligosaccharides and the GPI anchor.
- J. Zuegg and J. E. Gready (2000)
Glycobiology
10, 959-974
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- Follicular Dendritic Cells and Dissemination of Creutzfeldt-Jakob Disease.
- L. Manuelidis, I. Zaitsev, P. Koni, Z. Yun Lu, R. A. Flavell, and W. Fritch (2000)
J. Virol.
74, 8614-8622
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- Creutzfeldt-Jakob disease with a novel four extra-repeat insertional mutation in the PrP gene.
- G. Rossi, G. Giaccone, L. Giampaolo, S. Iussich, G. Puoti, M. Frigo, G. Cavaletti, L. Frattola, O. Bugiani, and F. Tagliavini (2000)
Neurology
55, 405-410
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- Adaptation and Selection of Prion Protein Strain Conformations following Interspecies Transmission of Transmissible Mink Encephalopathy.
- J. C. Bartz, R. A. Bessen, D. McKenzie, R. F. Marsh, and J. M. Aiken (2000)
J. Virol.
74, 5542-5547
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- Dominant-Negative Inhibition of Prion Formation Diminished by Deletion Mutagenesis of the Prion Protein.
- L. Zulianello, K. Kaneko, M. Scott, S. Erpel, D. Han, F. E. Cohen, and S. B. Prusiner (2000)
J. Virol.
74, 4351-4360
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- Successful Transmission of Three Mouse-Adapted Scrapie Strains to Murine Neuroblastoma Cell Lines Overexpressing Wild-Type Mouse Prion Protein.
- N. Nishida, D. A. Harris, D. Vilette, H. Laude, Y. Frobert, J. Grassi, D. Casanova, O. Milhavet, and S. Lehmann (2000)
J. Virol.
74, 320-325
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- Upregulation of the Genes Encoding Lysosomal Hydrolases, a Perforin-Like Protein, and Peroxidases in the Brains of Mice Affected with an Experimental Prion Disease.
- J. Kopacek, S. Sakaguchi, K. Shigematsu, N. Nishida, R. Atarashi, R. Nakaoke, R. Moriuchi, M. Niwa, and S. Katamine (2000)
J. Virol.
74, 411-417
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- Glycosylation differences between the normal and pathogenic prion protein isoforms.
- P. M. Rudd, T. Endo, C. Colominas, D. Groth, S. F. Wheeler, D. J. Harvey, M. R. Wormald, H. Serban, S. B. Prusiner, A. Kobata, et al. (1999)
PNAS
96, 13044-13049
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- PrP gene polymorphism and natural scrapie in Icelandic sheep.
- S. Thorgeirsdottir, S. Sigurdarson, H. M. Thorisson, G. Georgsson, and A. Palsdottir (1999)
J. Gen. Virol.
80, 2527-2534
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- Folding funnels and binding mechanisms.
- B. Ma, S. Kumar, C.-J. Tsai, and R. Nussinov (1999)
Protein Eng. Des. Sel.
12, 713-720
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- Sphingolipid Depletion Increases Formation of the Scrapie Prion Protein in Neuroblastoma Cells Infected with Prions.
- N. Naslavsky, H. Shmeeda, G. Friedlander, A. Yanai, A. H. Futerman, Y. Barenholz, and A. Taraboulos (1999)
J. Biol. Chem.
274, 20763-20771
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- A Delicate Balance: Homeostatic Control of Copper Uptake and Distribution.
- M. M. O. Peña, J. Lee, and D. J. Thiele (1999)
J. Nutr.
129, 1251-1260
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- Effectiveness of Polyene Antibiotics in Treatment of Transmissible Spongiform Encephalopathy in Transgenic Mice Expressing Syrian Hamster PrP Only in Neurons.
- R. Demaimay, R. Race, and B. Chesebro (1999)
J. Virol.
73, 3511-3513
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- Reversible Conversion of Monomeric Human Prion Protein Between Native and Fibrilogenic Conformations.
- G. S. Jackson, L. L. Hosszu, A. Power, A. F. Hill, J. Kenney, H. Saibil, C. J. Craven, J. P. Waltho, A. R. Clarke, and J. Collinge (1999)
Science
283, 1935-1937
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- Molecular Genetics of Transmissible Spongiform Encephalopathies.
- C. Weissmann (1999)
J. Biol. Chem.
274, 3-6
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- Expression of Cellular Prion Protein in Activated Hepatic Stellate Cells.
- K. Ikeda, N. Kawada, Y. Q. Wang, H. Kadoya, K. Nakatani, M. Sato, and K. Kaneda (1998)
Am. J. Pathol.
153, 1695-1700
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