Prion diseases and the BSE crisis.

Bovine spongiform encephalopathy (BSE) and human Creutzfeldt-Jakob disease (CJD) are among the most notable central nervous system degenerative disorders caused by prions. CJD may present as a sporadic, genetic, or infectious illness. Prions are transmissible particles that are devoid of nucleic acid and seem to be composed exclusively of a modified protein (PrPSc). The normal, cellular prion protein (PrPC) is converted into PrPSc through a posttranslational process during which it acquires a high beta-sheet content. It is thought that BSE is a result of cannibalism in which faulty industrial practices produced prion-contaminated feed for cattle. There is now considerable concern that bovine prions may have been passed to humans, resulting in a new form of CJD.

[1]  F. Cohen,et al.  Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation. , 1997, Proceedings of the National Academy of Sciences of the United States of America.

[2]  V. Coustou,et al.  The protein product of the het-s heterokaryon incompatibility gene of the fungus Podospora anserina behaves as a prion analog. , 1997, Proceedings of the National Academy of Sciences of the United States of America.

[3]  K Wüthrich,et al.  NMR characterization of the full‐length recombinant murine prion protein, mPrP(23–231) , 1997, FEBS letters.

[4]  S. Paushkin,et al.  In vitro propagation of the prion-like state of yeast Sup35 protein. , 1997, Science.

[5]  S. Lindquist,et al.  Self-Seeded Fibers Formed by Sup35, the Protein Determinant of [PSI +], a Heritable Prion-like Factor of S. cerevisiae , 1997, Cell.

[6]  N. Hunter,et al.  Is scrapie solely a genetic disease? , 1997, Nature.

[7]  G. Friedlander,et al.  Characterization of Detergent-insoluble Complexes Containing the Cellular Prion Protein and Its Scrapie Isoform* , 1997, The Journal of Biological Chemistry.

[8]  N. Ishiguro,et al.  Sensitive enzyme-linked immunosorbent assay for detection of PrP(Sc) in crude tissue extracts from scrapie-affected mice. , 1997, Journal of virological methods.

[9]  N. Hunter,et al.  Association between natural scrapie and PrP genotype in a flock of Suffolk sheep in Scotland , 1997, Veterinary Record.

[10]  J. Rossier,et al.  Transmission of the BSE Agent to Mice in the Absence of Detectable Abnormal Prion Protein , 1997, Science.

[11]  R. G. Will,et al.  Predicting the CJD epidemic in humans , 1997, Nature.

[12]  R. Carp,et al.  Scrapie strains retain their distinctive characteristics following passages of homogenates from different brain regions and spleen. , 1997, The Journal of general virology.

[13]  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.

[14]  S. Prusiner,et al.  Evidence for the Conformation of the Pathologic Isoform of the Prion Protein Enciphering and Propagating Prion Diversity , 1996, Science.

[15]  Y. Chernoff,et al.  Genesis and variability of [PSI] prion factors in Saccharomyces cerevisiae. , 1996, Genetics.

[16]  C. Gibbs,et al.  The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. , 1996, The New England journal of medicine.

[17]  R. Ridley,et al.  To what extent is strain variation evidence for an independent genome in the agent of the transmissible spongiform encephalopathies? , 1996, Neurodegeneration : a journal for neurodegenerative disorders, neuroprotection, and neuroregeneration.

[18]  Y. Agid,et al.  Creutzfeldt-Jakob disease from contaminated growth hormone extracts in France , 1996, Neurology.

[19]  C. Donnelly,et al.  Transmission dynamics and epidemiology of BSE in British cattle , 1996, Nature.

[20]  S. Prusiner,et al.  Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. , 1996, Genes & development.

[21]  R. Riek,et al.  NMR structure of the mouse prion protein domain PrP(121–231) , 1996, Nature.

[22]  J. Hauw,et al.  BSE transmission to macaques , 1996, Nature.

[23]  J Q Trojanowski,et al.  Molecular basis of phenotypic variability in sporadc creudeldt‐jakob disease , 1996, Annals of neurology.

[24]  F. Cohen,et al.  High-level expression and characterization of a purified 142-residue polypeptide of the prion protein. , 1996, Biochemistry.

[25]  David C. Krakauer,et al.  Phylogenesis of prion protein , 1996, Nature.

[26]  T. Noda,et al.  Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene , 1996, Nature.

[27]  P. Pergami,et al.  Semipreparative chromatographic method to purify the normal cellular isoform of the prion protein in nondenatured form. , 1996, Analytical biochemistry.

[28]  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.

[29]  A. Aguzzi,et al.  Prion protein (PrP) with amino‐proximal deletions restoring susceptibility of PrP knockout mice to scrapie. , 1996, The EMBO journal.

[30]  F. Cohen,et al.  Prion protein structure and scrapie replication: theoretical, spectroscopic, and genetic investigations. , 1996, Cold Spring Harbor symposia on quantitative biology.

[31]  S. Love,et al.  Sporadic Creutzfeldt-Jakob disease in a 18-year-old in the UK , 1995, The Lancet.

[32]  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.

[33]  D. Knowles,et al.  Experimental infection of cattle with the agents of transmissible mink encephalopathy and scrapie. , 1995, Journal of comparative pathology.

[34]  S. Prusiner,et al.  Complete Penetrance of Creutzfeldt-Jakob Disease in Libyan Jews Carrying the E200K Mutation in the Prion Protein Gene , 1995, Molecular medicine.

[35]  Fred E. Cohen,et al.  Conformational Transformations in Peptides Containing Two Putative α-Helices of the Prion Protein , 1995 .

[36]  P. Lansbury,et al.  Non-genetic propagation of strain-specific properties of scrapie prion protein , 1995, Nature.

[37]  D. Harris,et al.  Glycolipid-anchored proteins in neuroblastoma cells form detergent- resistant complexes without caveolin , 1995, The Journal of cell biology.

[38]  M. Smits,et al.  Identification of five allelic variants of the sheep PrP gene and their association with natural scrapie. , 1995, The Journal of general virology.

[39]  N. Hunter,et al.  Frequencies of PrP gene variants in healthy cattle and cattle with BSE in Scotland , 1994, Veterinary Record.

[40]  S. Prusiner,et al.  Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. , 1994, Proceedings of the National Academy of Sciences of the United States of America.

[41]  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.

[42]  J. Chapman,et al.  The risk of developing Creutzfeldt‐Jakob disease in subjects with the PRNP gene codon 200 point mutation , 1994, Neurology.

[43]  M. Palmer,et al.  Prion protein is necessary for normal synaptic function , 1994, Nature.

[44]  S. Prusiner,et al.  Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice. , 1994, Proceedings of the National Academy of Sciences of the United States of America.

[45]  R J Fletterick,et al.  Structural clues to prion replication. , 1994, Science.

[46]  R. Wickner,et al.  [URE3] as an altered URE2 protein: evidence for a prion analog in Saccharomyces cerevisiae. , 1994, Science.

[47]  S. Prusiner,et al.  Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie. , 1994, Genes & development.

[48]  B. Crain,et al.  Creutzfeldt-Jakob disease in a pregnant woman with an implanted dura mater graft. , 1994, Neurosurgery.

[49]  B. Ghetti,et al.  Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism. , 1994, Proceedings of the National Academy of Sciences of the United States of America.

[50]  H. Fraser,et al.  Transmission of bovine spongiform encephalopathy and scrapie to mice. , 1992, The Journal of general virology.

[51]  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.

[52]  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.

[53]  J. Ott,et al.  Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD). , 1993, American journal of human genetics.

[54]  P. Roller,et al.  Conformational transitions, dissociation, and unfolding of scrapie amyloid (prion) protein. , 1993, The Journal of biological chemistry.

[55]  C. Lueck,et al.  Creutzfeldt-Jakob disease and lyophilised dura mater grafts: report of two cases. , 1993, Journal of neurology, neurosurgery, and psychiatry.

[56]  A. Aguzzi,et al.  Mice devoid of PrP are resistant to scrapie , 1993, Cell.

[57]  S. Prusiner,et al.  Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes , 1993, Cell.

[58]  N. Hunter,et al.  Swaledale sheep affected by natural scrapie differ significantly in PrP genotype frequencies from healthy sheep and those selected for reduced incidence of scrapie. , 1993, The Journal of general virology.

[59]  R. Ridley,et al.  Experimental transmission of BSE and scrapie to the common marmoset , 1993, Veterinary Record.

[60]  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.

[61]  H Serban,et al.  Immunologic and molecular biologic studies of prion proteins in bovine spongiform encephalopathy. , 1993, The Journal of infectious diseases.

[62]  R. Fletterick,et al.  Perturbation of the secondary structure of the scrapie prion protein under conditions that alter infectivity. , 1993, Proceedings of the National Academy of Sciences of the United States of America.

[63]  F. Cohen,et al.  Predicted alpha-helical regions of the prion protein when synthesized as peptides form amyloid. , 1992, Proceedings of the National Academy of Sciences of the United States of America.

[64]  J. Martín,et al.  Analysis of the prion protein gene in thalamic dementia , 1992, Neurology.

[65]  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.

[66]  H. Fraser,et al.  Transmission of bovine spongiform encephalopathy and scrapie to mice , 2007 .

[67]  P. Brown,et al.  "Friendly fire" in medicine: hormones, homografts, and Creutzfeldt-Jakob disease , 1992, The Lancet.

[68]  S. Prusiner,et al.  Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters. , 1992, Genes & development.

[69]  C. Frith,et al.  Inherited prion disease with 144 base pair gene insertion. 1. Genealogical and molecular studies. , 1992, Brain : a journal of neurology.

[70]  W D Hueston,et al.  Bovine spongiform encephalopathy: case-control studies of calf feeding practices and meat and bonemeal inclusion in proprietary concentrates. , 1992, Research in veterinary science.

[71]  S. Prusiner,et al.  Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein , 1992, Nature.

[72]  S. Prusiner,et al.  Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene , 1992, Nature Genetics.

[73]  K. Taylor The control of bovine spongiform encephalopathy in Great Britain , 1991, Veterinary Record.

[74]  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.

[75]  C. Weissmann,et al.  A 'unified theory' of prion propagation , 1991, Nature.

[76]  B. Caughey,et al.  Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy. , 1991, Biochemistry.

[77]  S. Prusiner,et al.  Molecular biology of prion diseases , 1991, Science.

[78]  H. Budka,et al.  Prion protein mutation in family first reported by Gerstmann, Sträussler, and Scheinker , 1991, The Lancet.

[79]  S. Prusiner,et al.  Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. , 1991, The New England journal of medicine.

[80]  J W Wilesmith,et al.  Bovine spongiform encephalopathy: epidemiological studies on the origin , 1991, Veterinary Record.

[81]  H. Fraser,et al.  The disease characteristics of different strains of scrapie in Sinc congenic mouse lines: implications for the nature of the agent and host control of pathogenesis. , 1991, The Journal of general virology.

[82]  N. Hunter,et al.  Different forms of the bovine PrP gene have five or six copies of a short, G-C-rich element within the protein-coding exon. , 1991, The Journal of general virology.

[83]  S. Prusiner,et al.  Spontaneous neurodegeneration in transgenic mice with mutant prion protein , 1990, Science.

[84]  Stephen J. DeArmond,et al.  Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication , 1990, Cell.

[85]  S. Prusiner,et al.  RAPID DETECTION OF CREUTZFELDT-JAKOB DISEASE AND SCRAPIE PRION PROTEINS: 88 , 1990 .

[86]  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.

[87]  Stephen J. DeArmond,et al.  Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques , 1989, Cell.

[88]  W. Brown,et al.  Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker's syndrome , 1989, Experimental Neurology.

[89]  S. Prusiner,et al.  Diversity of oligosaccharide structures linked to asparagines of the scrapie prion protein. , 1989, Biochemistry.

[90]  L. Hood,et al.  Asparagine-linked glycosylation of the scrapie and cellular prion proteins. , 1989, Archives of biochemistry and biophysics.

[91]  Y. Sakaki,et al.  Pro----leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Sträussler syndrome. , 1989, Biochemical and biophysical research communications.

[92]  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.

[93]  Jurg Ott,et al.  Linkage of a prion protein missense variant to Gerstmann–Sträussler syndrome , 1989, Nature.

[94]  M. Lovett,et al.  Genetics and polymorphism of the mouse prion gene complex: control of scrapie incubation time , 1988, Molecular and cellular biology.

[95]  G. Multhaup,et al.  Fibrils from brains of cows with new cattle disease contain scrapie-associated protein , 1988, Nature.

[96]  L. Hood,et al.  Purification and properties of the cellular and scrapie hamster prion proteins. , 1988, European journal of biochemistry.

[97]  S. Cousens,et al.  Creutzfeldt-Jakob disease in England and Wales, 1980-1984: a case-control study of potential risk factors. , 1988, Journal of neurology, neurosurgery, and psychiatry.

[98]  G. Bock,et al.  Novel infectious agents and the central nervous system , 1988 .

[99]  S. Prusiner,et al.  Distinct prion proteins in short and long scrapie incubation period mice , 1987, Cell.

[100]  P. Castaigne,et al.  The epidemiology of Creutzfeldt‐Jakob disease , 1987, Neurology.

[101]  A. Dickinson,et al.  Biological evidence that scrapie agent has an independent genome. , 1987, The Journal of general virology.

[102]  S. Prusiner,et al.  Prions are novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease. , 1986, BioEssays : news and reviews in molecular, cellular and developmental biology.

[103]  S. Prusiner,et al.  Linkage of prion protein and scrapie incubation time genes , 1986, Cell.

[104]  Ruedi Aebersold,et al.  A cellular gene encodes scrapie PrP 27-30 protein , 1985, Cell.

[105]  S. Prusiner,et al.  Scrapie prions aggregate to form amyloid-like birefringent rods , 1983, Cell.

[106]  R. Kimberlin Reflections on the nature of scrapie agent , 1982 .

[107]  R. Kennedy,et al.  Natural infection of Suffolk sheep with scrapie virus. , 1982, The Journal of infectious diseases.

[108]  S. Prusiner,et al.  Kuru with incubation periods exceeding two decades , 1982, Annals of neurology.

[109]  S. Prusiner,et al.  Measurement of the scrapie agent using an incubation time interval assay , 1982, Annals of neurology.

[110]  C. Masters,et al.  Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies. , 1981, Brain : a journal of neurology.

[111]  Stanley B. Prusiner,et al.  Slow transmissible diseases of the nervous system , 1979 .

[112]  J. Winn,et al.  Brain , 1878, The Lancet.

[113]  D. Gajdusek Unconventional viruses and the origin and disappearance of kuru. , 1977, Science.

[114]  R. Kimberlin,et al.  Characteristics of a short incubation model of scrapie in the golden hamster. , 1977, The Journal of general virology.

[115]  W. J. Brown,et al.  Familial neurological disease associated with spongiform encephalopathy. , 1976, Archives of neurology.

[116]  J. Brody,et al.  Creutzfeldt-Jakob disease: a case-control study. , 1973, American journal of epidemiology.

[117]  A. Dickinson,et al.  A comparison of some biological characteristics of the mouse-passaged scrapie agents, 22A and ME7. , 1969, Genetical research.

[118]  M. Krigman The Central Nervous System: Some Experimental Models of Neurological Diseases , 1969 .

[119]  I. Pattison,et al.  Modification of a strain of mouse-adapted scrapie by passage through rats. , 1968, Research in veterinary science.

[120]  J. Mathews,et al.  Kuru and cannibalism. , 1968, Lancet.

[121]  H. Fraser,et al.  The sequential development of the brain lesion of scrapie in three strains of mice. , 1968, Journal of comparative pathology.

[122]  H. Fraser,et al.  Identification of a gene which controls the incubation period of some strains of scrapie agent in mice. , 1968, Journal of comparative pathology.

[123]  R. Hornabrook Kuru--a subacute cerebellar degeneration. The natural history and clinical features. , 1968, Brain : a journal of neurology.

[124]  C. Eklund,et al.  Pathogenesis of scrapie virus infection in the mouse. , 1967, The Journal of infectious diseases.

[125]  H. Parry,et al.  Scrapie: A transmissible and hereditary disease of sheep , 1962, Heredity.

[126]  Sarah Mae Sincero Heredity , 1875, Nature.