Deadly Conformations—Protein Misfolding in Prion Disease
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[1] P. MartínezMartín. [A new variant of Creutzfeldt-Jakob disease]. , 1997 .
[2] Christopher M. Dobson,et al. Instability, unfolding and aggregation of human lysozyme variants underlying amyloid fibrillogenesis , 1997, Nature.
[3] F. Cohen,et al. Recombinant scrapie-like prion protein of 106 amino acids is soluble. , 1996, Proceedings of the National Academy of Sciences of the United States of America.
[4] S. Prusiner,et al. Evidence for the Conformation of the Pathologic Isoform of the Prion Protein Enciphering and Propagating Prion Diversity , 1996, Science.
[5] A. Aguzzi,et al. Normal host prion protein (PrPC) is required for scrapie spread within the central nervous system. , 1996, Proceedings of the National Academy of Sciences of the United States of America.
[6] Andrew F. Hill,et al. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD , 1996, Nature.
[7] A. Aguzzi,et al. A suspicious signature , 1996, Nature.
[8] C. Donnelly,et al. Transmission dynamics and epidemiology of BSE in British cattle , 1996, Nature.
[9] Louise C. Serpell,et al. Synchrotron X-ray studies suggest that the core of the transthyretin amyloid fibril is a continuous β-sheet helix , 1996 .
[10] J R Glover,et al. Support for the Prion Hypothesis for Inheritance of a Phenotypic Trait in Yeast , 1996, Science.
[11] S. Prusiner,et al. Solid‐state NMR studies of the prion protein H1 fragment , 1996, Protein science : a publication of the Protein Society.
[12] S. Prusiner,et al. Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. , 1996, Genes & development.
[13] R. Riek,et al. NMR structure of the mouse prion protein domain PrP(121–231) , 1996, Nature.
[14] J. Hauw,et al. BSE transmission to macaques , 1996, Nature.
[15] S. Paushkin,et al. Propagation of the yeast prion‐like [psi+] determinant is mediated by oligomerization of the SUP35‐encoded polypeptide chain release factor. , 1996, The EMBO journal.
[16] F. Cohen,et al. Prion diseases of humans and animals , 1996 .
[17] J. Kelly,et al. The acid-mediated denaturation pathway of transthyretin yields a conformational intermediate that can self-assemble into amyloid. , 1996, Biochemistry.
[18] T. Noda,et al. Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene , 1996, Nature.
[19] A. Hofman,et al. A new variant of Creutzfeldt-Jakob disease in the UK , 1996, The Lancet.
[20] R. Nicoll,et al. Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus. , 1996, Proceedings of the National Academy of Sciences of the United States of America.
[21] A. Aguzzi,et al. Prion protein (PrP) with amino‐proximal deletions restoring susceptibility of PrP knockout mice to scrapie. , 1996, The EMBO journal.
[22] J. Kelly,et al. Alternative conformations of amyloidogenic proteins govern their behavior. , 1996, Current opinion in structural biology.
[23] C. Locht,et al. Immune system-dependent and -independent replication of the scrapie agent , 1996, Journal of virology.
[24] A. Aguzzi,et al. Normal host prion protein necessary for scrapie-induced neurotoxicity , 1996, Nature.
[25] P. Lansbury,et al. Aggregates of scrapie-associated prion protein induce the cell-free conversion of protease-sensitive prion protein to the protease-resistant state. , 1995, Chemistry & biology.
[26] C. Mineo,et al. A detergent-free method for purifying caveolae membrane from tissue culture cells. , 1995, Proceedings of the National Academy of Sciences of the United States of America.
[27] F. Cohen,et al. Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein , 1995, Cell.
[28] R. Wickner,et al. Prion-Inducing Domain of Yeast Ure2p and Protease Resistance of Ure2p in Prion-Containing Cells , 1995, Science.
[29] P. Lansbury,et al. Non-genetic propagation of strain-specific properties of scrapie prion protein , 1995, Nature.
[30] S W Liebman,et al. Role of the chaperone protein Hsp104 in propagation of the yeast prion-like factor [psi+]. , 1995, Science.
[31] Richard N. Sifers,et al. Defective protein folding as a cause of disease , 1995, Nature Structural Biology.
[32] P. Lansbury,et al. Species specificity in the cell-free conversion of prion protein to protease-resistant forms: a model for the scrapie species barrier. , 1995, Proceedings of the National Academy of Sciences of the United States of America.
[33] S. Prusiner,et al. Etiology and pathogenesis of prion diseases. , 1995, The American journal of pathology.
[34] S. Prusiner,et al. Cholesterol depletion and modification of COOH-terminal targeting sequence of the prion protein inhibit formation of the scrapie isoform [published erratum appears in J Cell Biol 1995 Jul;130(2):501] , 1995, The Journal of cell biology.
[35] M. Palmer,et al. Rescue of neurophysiological phenotype seen in PrP null mice by transgene encoding human prion protein , 1995, Nature Genetics.
[36] R. Marsh,et al. Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy , 1994, Journal of virology.
[37] Susan Lindquist,et al. Protein disaggregation mediated by heat-shock protein Hspl04 , 1994, Nature.
[38] D. Baker,et al. Influenza hemagglutinin: kinetic control of protein function. , 1994, Structure.
[39] S. Prusiner,et al. Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. , 1994, Proceedings of the National Academy of Sciences of the United States of America.
[40] P. Lansbury,et al. Cell-free formation of protease-resistant prion protein , 1994, Nature.
[41] M. Palmer,et al. Prion protein is necessary for normal synaptic function , 1994, Nature.
[42] D A Agard,et al. Kinetics versus thermodynamics in protein folding. , 1994, Biochemistry.
[43] R J Fletterick,et al. Structural clues to prion replication. , 1994, Science.
[44] P. Roller,et al. Thermal stability and conformational transitions of scrapie amyloid (prion) protein correlate with infectivity , 1993, Protein science : a publication of the Protein Society.
[45] R J Fletterick,et al. Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins. , 1993, Proceedings of the National Academy of Sciences of the United States of America.
[46] S. Prusiner,et al. Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies. , 1993, Proceedings of the National Academy of Sciences of the United States of America.
[47] Anonymous,et al. Review: , 2019 .
[48] R. Bowler,et al. Three scrapie prion isolates exhibit different accumulation patterns of the prion protein scrapie isoform. , 1993, Proceedings of the National Academy of Sciences of the United States of America.
[49] A. Aguzzi,et al. Mice devoid of PrP are resistant to scrapie , 1993, Cell.
[50] P. Lansbury,et al. Seeding “one-dimensional crystallization” of amyloid: A pathogenic mechanism in Alzheimer's disease and scrapie? , 1993, Cell.
[51] S. Prusiner,et al. Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes , 1993, Cell.
[52] S. Prusiner,et al. Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cells. , 1993, Proceedings of the National Academy of Sciences of the United States of America.
[53] L. Hood,et al. Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing. , 1993, Biochemistry.
[54] S. Prusiner,et al. Regional mapping of prion proteins in brain. , 1992, Proceedings of the National Academy of Sciences of the United States of America.
[55] S. Prusiner,et al. Evidence for synthesis of scrapie prion proteins in the endocytic pathway. , 1992, The Journal of biological chemistry.
[56] S. Prusiner,et al. Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters. , 1992, Genes & development.
[57] T. Kitamoto,et al. The sequential development of abnormal prion protein accumulation in mice with Creutzfeldt-Jakob disease. , 1992, The American journal of pathology.
[58] S. Prusiner,et al. Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein , 1992, Nature.
[59] R. Marsh,et al. Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent , 1992, Journal of virology.
[60] S. Prusiner,et al. Further analysis of nucleic acids in purified scrapie prion preparations by improved return refocusing gel electrophoresis. , 1992, The Journal of general virology.
[61] Deborah A. Brown,et al. Sorting of GPI-anchored proteins to glycolipid-enriched membrane subdomains during transport to the apical cell surface , 1992, Cell.
[62] C. Bouras,et al. Fatal familial insomnia , 1992, Neurology.
[63] K. Doh-ura,et al. Abnormal isoform of prion protein accumulates in follicular dendritic cells in mice with Creutzfeldt-Jakob disease , 1991, Journal of virology.
[64] G. J. Raymond,et al. The scrapie-associated form of PrP is made from a cell surface precursor that is both protease- and phospholipase-sensitive. , 1991, The Journal of biological chemistry.
[65] B. Caughey,et al. Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy. , 1991, Biochemistry.
[66] John Collinge,et al. Homozygous prion protein genotype predisposes to sporadic Creutzfeldt–Jakob disease , 1991, Nature.
[67] S. Prusiner,et al. Molecular biology of prion diseases , 1991, Science.
[68] R. Carp,et al. Variation in the characteristics of 10 mouse-passaged scrapie lines derived from five scrapie-positive sheep. , 1991, The Journal of general virology.
[69] S. Prusiner,et al. Spontaneous neurodegeneration in transgenic mice with mutant prion protein , 1990, Science.
[70] Stephen J. DeArmond,et al. Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication , 1990, Cell.
[71] S. Prusiner,et al. Acquisition of protease resistance by prion proteins in scrapie-infected cells does not require asparagine-linked glycosylation. , 1990, Proceedings of the National Academy of Sciences of the United States of America.
[72] S. Prusiner,et al. Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells , 1990, The Journal of cell biology.
[73] R. Ishima,et al. Termini of Salmonella flagellin are disordered and become organized upon polymerization into flagellar filament. , 1990, Journal of molecular biology.
[74] Stephen J. DeArmond,et al. Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques , 1989, Cell.
[75] H. Fraser,et al. The genomic identity of different strains of mouse scrapie is expressed in hamsters and preserved on reisolation in mice. , 1989, The Journal of general virology.
[76] Jurg Ott,et al. Linkage of a prion protein missense variant to Gerstmann–Sträussler syndrome , 1989, Nature.
[77] B. Chesebro,et al. Prion protein biosynthesis in scrapie-infected and uninfected neuroblastoma cells , 1989, Journal of virology.
[78] D. Gajdusek. Transmissible and non-transmissible amyloidoses: autocatalytic post-translational conversion of host precursor proteins to β-pleated sheet configurations , 1988, Journal of Neuroimmunology.
[79] S. Prusiner,et al. Immunoaffinity purification and neutralization of scrapie prion infectivity. , 1988, Proceedings of the National Academy of Sciences of the United States of America.
[80] S. Prusiner,et al. Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins , 1988, Journal of virology.
[81] S. Prusiner,et al. Distinct prion proteins in short and long scrapie incubation period mice , 1987, Cell.
[82] Stanley B. Prusiner,et al. Scrapie prion protein contains a phosphatidylinositol glycolipid , 1987, Cell.
[83] G. Multhaup,et al. The major polypeptide of scrapie‐associated fibrils (SAF) has the same size, charge distribution and N‐terminal protein sequence as predicted for the normal brain protein (PrP). , 1986, The EMBO journal.
[84] S. Prusiner,et al. Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene , 1986, Cell.
[85] S. Prusiner,et al. Separation and properties of cellular and scrapie prion proteins. , 1986, Proceedings of the National Academy of Sciences of the United States of America.
[86] B. Chesebro,et al. Identification of scrapie prion protein-specific mRNA in scrapie-infected and uninfected brain , 1985, Nature.
[87] Ruedi Aebersold,et al. A cellular gene encodes scrapie PrP 27-30 protein , 1985, Cell.
[88] L. Hood,et al. Purification and structural studies of a major scrapie prion protein , 1984, Cell.
[89] S. Prusiner,et al. Scrapie prions aggregate to form amyloid-like birefringent rods , 1983, Cell.
[90] H. Gelderblom,et al. Scrapie infectivity, fibrils and low molecular weight protein , 1983, Nature.
[91] S. Hall,et al. Pathogenesis of mouse scrapie Evidence for direct neural spread of infection to the CNS after injection of sciatic nerve , 1983, Journal of the Neurological Sciences.
[92] S. Prusiner,et al. Identification of a protein that purifies with the scrapie prion. , 1982, Science.
[93] S. Prusiner,et al. Further purification and characterization of scrapie prions. , 1982, Biochemistry.
[94] S. Prusiner. Novel proteinaceous infectious particles cause scrapie. , 1982, Science.
[95] S. Prusiner,et al. Molecular properties, partial purification, and assay by incubation period measurements of the hamster scrapie agent. , 1980, Biochemistry.
[96] R. Kimberlin,et al. Pathogenesis of mouse scrapie: dynamics of agent replication in spleen, spinal cord and brain after infection by different routes. , 1979, Journal of comparative pathology.
[97] D. Gajdusek. Unconventional viruses and the origin and disappearance of kuru. , 1977, Science.
[98] H. Fraser,et al. CEREBRAL AMYLOIDOSIS IN SCRAPIE IN THE MOUSE: EFFECT OF AGENT STRAIN AND MOUSE GENOTYPE , 1976 .
[99] J. S. Orr,et al. CLAIMS FOR A DIATHERMY MACHINE , 1974 .
[100] R. Latarjet,et al. Inactivation of the Scrapie Agent by Near Monochromatic Ultraviolet Light , 1970, Nature.
[101] P. Daniel,et al. Creutzfeldt-Jakob Disease (Spongiform Encephalopathy): Transmission to the Chimpanzee , 1968, Science.
[102] S. Asakura. A kinetic study of in vitro polymerization of flagellin. , 1968, Journal of molecular biology.
[103] J. Morris,et al. Scrapie in Mice , 1968, Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine.
[104] J. Griffith,et al. Nature of the Scrapie Agent: Self-replication and Scrapie , 1967, Nature.
[105] TIKVAH ALPER,et al. Does the Agent of Scrapie Replicate without Nucleic Acid ? , 1967, Nature.
[106] C. Eklund,et al. Pathogenesis of scrapie virus infection in the mouse. , 1967, The Journal of infectious diseases.
[107] S. Asakura,et al. Salmonella flagella: in vitro reconstruction and over-all shapes of flagellar filaments. , 1966, Journal of molecular biology.
[108] M. Alpers,et al. Experimental Transmission of a Kuru-like Syndrome to Chimpanzees , 1966, Nature.
[109] T. Alper,et al. The exceptionally small size of the scrapie agent. , 1966, Biochemical and biophysical research communications.
[110] S. Asakura,et al. RECONSTITUTION OF BACTERIAL FLAGELLA IN VITRO. , 1964, Journal of molecular biology.
[111] D. Gajdusek,et al. Kuru; clinical, pathological and epidemiological study of an acute progressive degenerative disease of the central nervous system among natives of the Eastern Highlands of New Guinea. , 1959, The American journal of medicine.
[112] K. Satoh,et al. KURU , 1957, Psychological Medicine.
[113] Gordon Ws. Advances in veterinary research. , 1946 .
[114] J. Gerstmann,et al. Über eine eigenartige hereditär- familiäre Erkrankung des Zentralnervensystems , 1935 .
[115] R. Riek. NMR structure of the mouse prion protein , 1998 .
[116] A. Aguzzi,et al. The role of PrP in pathogenesis of experimental scrapie. , 1996, Cold Spring Harbor symposia on quantitative biology.
[117] De Armond. Three scrapie prion isolates exhibit different accumulation patterns of the prion protein scrapie isoform. , 1993 .
[118] S. Prusiner,et al. IMMUNOAFFINITY PURIFICATION AND NEUTRALIZATION OF SCRAPIE PRIONS , 1988 .
[119] J. Szulmajster. Protein folding , 1988, Bioscience reports.
[120] H. Fraser,et al. Scrapie in mice. Agent-strain differences in the distribution and intensity of grey matter vacuolation. , 1973, Journal of comparative pathology.
[121] J. Griffith,et al. Self-replication and scrapie. , 1967, Nature.
[122] T. Hulland. Scrapie and Kuru. , 1960, The Canadian veterinary journal = La revue veterinaire canadienne.
[123] W. S. Gordon. Advances in veterinary research. , 1946, The Veterinary record.