Transmissible spongiform encephalopathies

[1]  A. Aguzzi,et al.  Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease. , 2003, The New England journal of medicine.

[2]  S. Prusiner,et al.  Trafficking of prion proteins through a caveolae-mediated endosomal pathway , 2003, The Journal of cell biology.

[3]  G. Forloni,et al.  Evaluation of Quinacrine Treatment for Prion Diseases , 2003, Journal of Virology.

[4]  S. Laboissiere,et al.  A prion protein epitope selective for the pathologically misfolded conformation , 2003, Nature Medicine.

[5]  J. Chabry,et al.  Specific inhibition of pathological prion protein accumulation by small interfering RNAs , 2003, Journal of Cell Science.

[6]  R. S. Stewart,et al.  Mutant PrP Is Delayed in Its Exit from the Endoplasmic Reticulum, but Neither Wild-type nor Mutant PrP Undergoes Retrotranslocation Prior to Proteasomal Degradation* , 2003, Journal of Biological Chemistry.

[7]  S. Prusiner,et al.  Copper coordination in the full-length, recombinant prion protein. , 2003, Biochemistry.

[8]  C. Masters,et al.  Novel prion protein insert mutation associated with prolonged neurodegenerative illness , 2003, Neurology.

[9]  Michael P H Stumpf,et al.  Balancing Selection at the Prion Protein Gene Consistent with Prehistoric Kurulike Epidemics , 2003, Science.

[10]  J. Collinge,et al.  Monoclonal antibodies inhibit prion replication and delay the development of prion disease , 2003, Nature.

[11]  J. H. Viles,et al.  Copper Binding to the Octarepeats of the Prion Protein , 2003, The Journal of Biological Chemistry.

[12]  J. Collinge,et al.  BSE prions propagate as either variant CJD‐like or sporadic CJD‐like prion strains in transgenic mice expressing human prion protein , 2002, The EMBO journal.

[13]  G. J. Raymond,et al.  Subclinical scrapie infection in a resistant species: persistence, replication, and adaptation of infectivity during four passages. , 2002, The Journal of infectious diseases.

[14]  J. Kaldor,et al.  Creutzfeldt–Jakob disease in Australia 1970–1999 , 2002, Neurology.

[15]  A. Ghani,et al.  Implications of BSE infection screening data for the scale of the British BSE epidemic and current European infection levels , 2002, Proceedings of the Royal Society of London. Series B: Biological Sciences.

[16]  N. Hunter,et al.  Transmission of prion diseases by blood transfusion. , 2002, The Journal of general virology.

[17]  S. Prusiner,et al.  Measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice , 2002, Nature Biotechnology.

[18]  S. Lindquist,et al.  Conversion of PrP to a Self-Perpetuating PrPSc-like Conformation in the Cytosol , 2002, Science.

[19]  S. Lindquist,et al.  Neurotoxicity and Neurodegeneration When PrP Accumulates in the Cytosol , 2002, Science.

[20]  C. Masters,et al.  Quinacrine does not prolong survival in a murine Creutzfeldt‐Jakob disease model , 2002, Annals of neurology.

[21]  H. Budka,et al.  Spontaneous mutations in the prion protein gene causing transmissible spongiform encephalopathy , 2002, Annals of neurology.

[22]  C. V. van Duijn,et al.  Sporadic Creutzfeldt–Jakob disease and surgery: A case–control study using community controls , 2002, Neurology.

[23]  A. Aguzzi,et al.  Incidence of Creutzfeldt-Jakob disease in Switzerland , 2002, The Lancet.

[24]  J. Kaldor,et al.  Creutzfeldt‐Jakob disease cluster in an Australian rural city , 2002, Annals of neurology.

[25]  P. Brown.  Drug therapy in human and experimental transmissible spongiform encephalopathy , 2002, Neurology.

[26]  R. Will,et al.  First hundred cases of variant Creutzfeldt-Jakob disease: retrospective case note review of early psychiatric and neurological features , 2002, BMJ : British Medical Journal.

[27]  W. K. Cullen,et al.  Naturally secreted oligomers of amyloid β protein potently inhibit hippocampal long-term potentiation in vivo , 2002, Nature.

[28]  Eliah Aronoff-Spencer,et al.  Molecular features of the copper binding sites in the octarepeat domain of the prion protein. , 2002, Biochemistry.

[29]  S. Prusiner,et al.  Prions in skeletal muscle , 2002, Proceedings of the National Academy of Sciences of the United States of America.

[30]  B. Chesebro,et al.  Conversion of raft associated prion protein to the protease‐resistant state requires insertion of PrP‐res (PrPSc) into contiguous membranes , 2002, The EMBO journal.

[31]  R. Sánchez-Valle,et al.  14-3-3 protein isoforms and atypical patterns of the 14-3-3 assay in the diagnosis of Creutzfeldt–Jakob disease , 2002, Neuroscience Letters.

[32]  A. Aguzzi,et al.  Chronic Subclinical Prion Disease Induced by Low-Dose Inoculum , 2002, Journal of Virology.

[33]  Witold K. Surewicz,et al.  Crystal structure of the human prion protein reveals a mechanism for oligomerization , 2002, Nature Structural Biology.

[34]  J. Wiltfang,et al.  Tau protein and 14-3-3 protein in the differential diagnosis of Creutzfeldt–Jakob disease , 2002, Neurology.

[35]  A. Valleron,et al.  Estimation of Epidemic Size and Incubation Time Based on Age Characteristics of vCJD in the United Kingdom , 2001, Science.

[36]  Terrance T. Kummer,et al.  Mutant Prion Proteins Are Partially Retained in the Endoplasmic Reticulum* , 2001, The Journal of Biological Chemistry.

[37]  G. J. Raymond,et al.  Long-Term Subclinical Carrier State Precedes Scrapie Replication and Adaptation in a Resistant Species: Analogies to Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease in Humans , 2001, Journal of Virology.

[38]  S. Cousens,et al.  Predictability of the UK Variant Creutzfeldt-Jakob Disease Epidemic , 2001, Science.

[39]  Albert Taraboulos,et al.  Proteasomes and ubiquitin are involved in the turnover of the wild‐type prion protein , 2001, The EMBO journal.

[40]  J. Walts.  Bovine spongiform encephalopathy case found in Japan , 2001, The Lancet.

[41]  D. Melton,et al.  Changing a single amino acid in the N‐terminus of murine PrP alters TSE incubation time across three species barriers , 2001, The EMBO journal.

[42]  F. Heppner,et al.  Prevention of Scrapie Pathogenesis by Transgenic Expression of Anti-Prion Protein Antibodies , 2001, Science.

[43]  A. Colchester,et al.  MRI of Creutzfeldt-Jakob disease: imaging features and recommended MRI protocol. , 2001, Clinical radiology.

[44]  C. Masters,et al.  Gerstmann–Sträussler–Scheinker syndrome,fatal familial insomnia, and kuru: a review ofthese less common human transmissiblespongiform encephalopathies , 2001, Journal of Clinical Neuroscience.

[45]  F E Cohen,et al.  Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease , 2001, Proceedings of the National Academy of Sciences of the United States of America.

[46]  B. Caughey,et al.  Reversibility of Scrapie-associated Prion Protein Aggregation* , 2001, The Journal of Biological Chemistry.

[47]  M. Enari,et al.  Scrapie prion protein accumulation by scrapie-infected neuroblastoma cells abrogated by exposure to a prion protein antibody , 2001, Proceedings of the National Academy of Sciences of the United States of America.

[48]  J. Collinge,et al.  Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay , 2001, The Lancet.

[49]  J. Collinge,et al.  Location and properties of metal-binding sites on the human prion protein , 2001, Proceedings of the National Academy of Sciences of the United States of America.

[50]  A. Bush,et al.  Copper and zinc binding modulates the aggregation and neurotoxic properties of the prion peptide PrP106-126. , 2001, Biochemistry.

[51]  J. Ironside,et al.  Use of 14–3–3 and other brain-specific proteins in CSF in the diagnosis of variant Creutzfeldt-Jakob disease , 2001, Journal of neurology, neurosurgery, and psychiatry.

[52]  S. Prusiner,et al.  Shattuck lecture--neurodegenerative diseases and prions. , 2001, The New England journal of medicine.

[53]  S. Priola,et al.  Efficient Conversion of Normal Prion Protein (PrP) by Abnormal Hamster PrP Is Determined by Homology at Amino Acid Residue 155 , 2001, Journal of Virology.

[54]  W A Rutala,et al.  Creutzfeldt-Jakob disease: recommendations for disinfection and sterilization. , 2001, Clinical infectious diseases : an official publication of the Infectious Diseases Society of America.

[55]  N. Hooper,et al.  Ablation of the metal ion-induced endocytosis of the prion protein by disease-associated mutation of the octarepeat region , 2001, Current Biology.

[56]  S. Prusiner,et al.  Immobilized prion protein undergoes spontaneous rearrangement to a conformation having features in common with the infectious form , 2001, The EMBO journal.

[57]  M. Alpers,et al.  Increased susceptibility to Kuru of carriers of the PRNP 129 methionine/methionine genotype. , 2001, The Journal of infectious diseases.

[58]  L. McShane,et al.  Iatrogenic Creutzfeldt–Jakob disease at the millennium , 2000, Neurology.

[59]  H. Budka,et al.  Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt–Jakob disease , 2000, Neurology.

[60]  P. Lantos,et al.  Species-barrier-independent prion replication in apparently resistant species. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[61]  K Wüthrich,et al.  NMR structure of the bovine prion protein. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[62]  B. Caughey,et al.  Lysosomotropic Agents and Cysteine Protease Inhibitors Inhibit Scrapie-Associated Prion Protein Accumulation , 2000, Journal of virology.

[63]  S. Cousens,et al.  Diagnosis of new variant Creutzfeldt‐Jakob disease , 2000, Annals of neurology.

[64]  F E Cohen,et al.  A synthetic peptide initiates Gerstmann-Sträussler-Scheinker (GSS) disease in transgenic mice. , 2000, Journal of molecular biology.

[65]  A. Aguzzi,et al.  Scrapie Pathogenesis in Subclinically Infected B-Cell-Deficient Mice , 1999, Journal of Virology.

[66]  P Brown,et al.  Classification of sporadic Creutzfeldt‐Jakob disease based on molecular and phenotypic analysis of 300 subjects , 1999, Annals of neurology.

[67]  S. Prusiner,et al.  Prion protein conformation in a patient with sporadic fatal insomnia. , 1999, The New England journal of medicine.

[68]  J. Collinge,et al.  Strain-specific prion-protein conformation determined by metal ions , 1999, Nature Cell Biology.

[69]  M. Enari,et al.  Infectivity of Scrapie Prions Bound to a Stainless Steel Surface , 1999, Molecular medicine.

[70]  J Collinge,et al.  Reversible conversion of monomeric human prion protein between native and fibrilogenic conformations. , 1999, Science.

[71]  C. Masters,et al.  Surgical treatment and risk of sporadic Creutzfeldt-Jakob disease: a case-control study , 1999, The Lancet.

[72]  J Collinge,et al.  Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples , 1999, The Lancet.

[73]  J. Laplanche,et al.  14-3-3 Protein, Neuron-Specific Enolase, and S-100 Protein in Cerebrospinal Fluid of Patients with Creutzfeldt-Jakob Disease , 1998, Dementia and Geriatric Cognitive Disorders.

[74]  A. Giese,et al.  Phenotypic variability in fatal familial insomnia (D178N-129M) genotype , 1998, Neurology.

[75]  P. Brown,et al.  Phenotype-genotype studies in kuru: implications for new variant Creutzfeldt-Jakob disease. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[76]  J. Aiken,et al.  Reversibility of Scrapie Inactivation Is Enhanced by Copper* , 1998, The Journal of Biological Chemistry.

[77]  A. Hofman,et al.  Descriptive epidemiology of Creutzfeldt‐Jakob disease in six european countries, 1993–1995 , 1998, Annals of neurology.

[78]  S. Prusiner,et al.  A transmembrane form of the prion protein in neurodegenerative disease. , 1998, Science.

[79]  D. Westaway,et al.  The cellular prion protein binds copper in vivo , 1997, Nature.

[80]  Andrew F. Hill,et al.  The same prion strain causes vCJD and BSE , 1997, Nature.

[81]  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.

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

[83]  S. Cousens,et al.  Sporadic Creutzfeldt-Jakob disease in the United Kingdom: analysis of epidemiological surveillance data for 1970-96 , 1997, BMJ.

[84]  E. Storey,et al.  The D178N (cis-129M) "fatal familial insomnia" mutation associated with diverse clinicopathologic phenotypes in an Australian kindred , 1997, Neurology.

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

[86]  Andrew F. Hill,et al.  Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD , 1996, Nature.

[87]  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.

[88]  R. Lathe,et al.  Genetic basis of Creutzfeldt-Jakob disease in the United Kingdom: a systematic analysis of predisposing mutations and allelic variation in the PRNP gene , 1996, Human Genetics.

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

[90]  A. Hofman,et al.  A new variant of Creutzfeldt-Jakob disease in the UK , 1996, The Lancet.

[91]  B. Steinhoff,et al.  Accuracy and reliability of periodic sharp wave complexes in Creutzfeldt-Jakob disease. , 1996, Archives of neurology.

[92]  P. Brown,et al.  First experimental transmission of fatal familial insomnia , 1995, Nature.

[93]  A. Garay,et al.  Clinical and genetic studies of fatal familial insomnia , 1995, Neurology.

[94]  P. Lansbury,et al.  Cell-free formation of protease-resistant prion protein , 1994, Nature.

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

[96]  D. Harris,et al.  A glycolipid-anchored prion protein is endocytosed via clathrin-coated pits , 1994, The Journal of cell biology.

[97]  P. Brown,et al.  Human spongiform encephalopathy: The national institutes of health series of 300 cases of experimentally transmitted disease , 1994, Annals of neurology.

[98]  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.

[99]  D. Perani,et al.  [18F]FDG PET in fatal familial insomnia , 1993, Neurology.

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

[101]  P Brown,et al.  Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism. , 1992, Science.

[102]  T. Crow,et al.  Inherited prion disease with 144 base pair gene insertion. 2. Clinical and pathological features. , 1992, Brain : a journal of neurology.

[103]  E. Sforza,et al.  Fatal familial insomnia , 1992, Neurology.

[104]  H. Y. Chen,et al.  Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene. , 1992, The New England journal of medicine.

[105]  G. J. Raymond,et al.  N-terminal truncation of the scrapie-associated form of PrP by lysosomal protease(s): implications regarding the site of conversion of PrP to the protease-resistant state , 1991, Journal of virology.

[106]  A. C. Fabian,et al.  Binary precursor for planet? , 1991, Nature.

[107]  A. Korczyn,et al.  Creutzfeldt-Jacob disease associated with the PRNP codon 200LYS mutation: An analysis of 45 families , 1991, European Journal of Epidemiology.

[108]  J. S. Miles,et al.  Prediction of debrisoquine phenotype , 1991, The Lancet.

[109]  P. Brown,et al.  Codon 178 mutation in ethnically diverse Creutzfeldt-Jakob disease families , 1991, The Lancet.

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

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

[112]  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.

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

[114]  S. Prusiner,et al.  Identification of a protein that purifies with the scrapie prion. , 1982, Science.

[115]  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.

[116]  Jonathan O. Harris,et al.  Creutzfeldt‐Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering , 1979, Annals of neurology.

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

[118]  M. Alpers,et al.  PRECLINICAL LESIONS AND THEIR PROGRESSION IN THE EXPERIMENTAL SPONGIFORM ENCEPHALOPATHIES (KURU AND CREUTZFELDT‐JAKOB DISEASE) IN PRIMATES , 1976, Journal of neuropathology and experimental neurology.

[119]  J. S. Orr,et al.  CLAIMS FOR A DIATHERMY MACHINE , 1974 .

[120]  M. Alpers,et al.  Experimental Transmission of a Kuru-like Syndrome to Chimpanzees , 1966, Nature.

[121]  W. Hadlow.  SCRAPIE AND KURU , 1959 .

[122]  D. Gajdusek,et al.  Degenerative disease of the central nervous system in New Guinea; the endemic occurrence of kuru in the native population. , 1957, The New England journal of medicine.

[123]  V. Zigas,et al.  KURU: CLINICAL STUDY OF A NEW SYNDROME RESEMBLING PARALYSIS AGITANS IN NATIVES OF THE EASTERN HIGHLANDS OF AUSTRALIAN NEW GUINEA , 1957, The Medical journal of Australia.

[124]  C. Watson.  A case of mesenteric vascular occlusion. , 1949, The Medical journal of Australia.

[125]  K. Stern.  SEVERE DEMENTIA ASSOCIATED WITH BILATERAL SYMMETRICAL DEGENERATION OF THE THALAMUS , 1939 .

[126]  D. Taylor.  Inactivation of transmissible degenerative encephalopathy agents: A review. , 2000, Veterinary journal.

[127]  J. Collinge,et al.  Protease-resistant prion protein produced in vitro lacks detectable infectivity. , 1999, The Journal of general virology.

[128]  M. Rogers,et al.  Clathrin-coated vesicles and detergent-resistant rafts in prion biology. Commentary , 1998 .

[129]  M. Alpers,et al.  The Natural Incubation Period of Kuru and the Episodes of Transmission in Three Clusters of Patients , 1984 .