Adriano Aguzzi

Summary

Affiliation: University Hospital
Country: Switzerland

Publications

  1. ncbi request reprint [The prion hypothesis and the human prion diseases]
    Adriano Aguzzi
    Universitatsspital, Universitat Zurich
    Berl Munch Tierarztl Wochenschr 115:91-8. 2002
  2. ncbi request reprint Positioning of follicular dendritic cells within the spleen controls prion neuroinvasion
    Marco Prinz
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nature 425:957-62. 2003
  3. ncbi request reprint Games played by rogue proteins in prion disorders and Alzheimer's disease
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Science 302:814-8. 2003
  4. ncbi request reprint Prions and peripheral nerves: a deadly rendezvous
    Mathias Heikenwalder
    Department of Pathology, Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    J Neurosci Res 85:2714-25. 2007
  5. ncbi request reprint The prion's elusive reason for being
    Adriano Aguzzi
    Institute of Neuropathology, University of Zurich, CH 8091 Zurich, Switzerland
    Annu Rev Neurosci 31:439-77. 2008
  6. pmc The comprehensive native interactome of a fully functional tagged prion protein
    Dorothea Rutishauser
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    PLoS ONE 4:e4446. 2009
  7. pmc Engulfment of cerebral apoptotic bodies controls the course of prion disease in a mouse strain-dependent manner
    Jan Kranich
    Institute of Neuropathology, University Hospital of Zurich, 8091 Zurich, Switzerland
    J Exp Med 207:2271-81. 2010
  8. pmc Functionally relevant domains of the prion protein identified in vivo
    Frank Baumann
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    PLoS ONE 4:e6707. 2009
  9. pmc Aerosols transmit prions to immunocompetent and immunodeficient mice
    Johannes Haybaeck
    Department of Pathology, Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
    PLoS Pathog 7:e1001257. 2011
  10. pmc Unexpected tolerance of alpha-cleavage of the prion protein to sequence variations
    José B Oliveira-Martins
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    PLoS ONE 5:e9107. 2010

Detail Information

Publications109 found, 100 shown here

  1. ncbi request reprint [The prion hypothesis and the human prion diseases]
    Adriano Aguzzi
    Universitatsspital, Universitat Zurich
    Berl Munch Tierarztl Wochenschr 115:91-8. 2002
    ..This overview summarizes some current hypotheses about the nature of the infectious agent and about the pathogenesis of the damage of the central nervous system...
  2. ncbi request reprint Positioning of follicular dendritic cells within the spleen controls prion neuroinvasion
    Marco Prinz
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nature 425:957-62. 2003
    ..This suggests that prion neuroimmune transition occurs between FDCs and sympathetic nerves, and relative positioning of FDCs and nerves controls the efficiency of peripheral prion infection...
  3. ncbi request reprint Games played by rogue proteins in prion disorders and Alzheimer's disease
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Science 302:814-8. 2003
    ..There is reason to expect that prion research will profit from advances in the understanding of AD, and vice versa...
  4. ncbi request reprint Prions and peripheral nerves: a deadly rendezvous
    Mathias Heikenwalder
    Department of Pathology, Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    J Neurosci Res 85:2714-25. 2007
    ..Although research in the past has accumulated knowledge on prion progression from the periphery to the brain, we are far from understanding the molecular mechanisms of neuronal prion transport...
  5. ncbi request reprint The prion's elusive reason for being
    Adriano Aguzzi
    Institute of Neuropathology, University of Zurich, CH 8091 Zurich, Switzerland
    Annu Rev Neurosci 31:439-77. 2008
    ..Here we review the salient traits of those diseases ascribed to improper behavior of the prion protein and highlight how the physiological functions of PrP(C) may help explain the toxic phenotypes observed in prion disease...
  6. pmc The comprehensive native interactome of a fully functional tagged prion protein
    Dorothea Rutishauser
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    PLoS ONE 4:e4446. 2009
    ..Selected PrP(C) interactors were validated using independent methods. Several of these proteins appear to exert functions in axomyelinic maintenance...
  7. pmc Engulfment of cerebral apoptotic bodies controls the course of prion disease in a mouse strain-dependent manner
    Jan Kranich
    Institute of Neuropathology, University Hospital of Zurich, 8091 Zurich, Switzerland
    J Exp Med 207:2271-81. 2010
    ....
  8. pmc Functionally relevant domains of the prion protein identified in vivo
    Frank Baumann
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    PLoS ONE 4:e6707. 2009
    ....
  9. pmc Aerosols transmit prions to immunocompetent and immunodeficient mice
    Johannes Haybaeck
    Department of Pathology, Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
    PLoS Pathog 7:e1001257. 2011
    ..This previously unappreciated risk for airborne prion transmission may warrant re-thinking on prion biosafety guidelines in research and diagnostic laboratories...
  10. pmc Unexpected tolerance of alpha-cleavage of the prion protein to sequence variations
    José B Oliveira-Martins
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    PLoS ONE 5:e9107. 2010
    ..These results suggest that alpha-cleavage is executed by an alpha-PrPase whose activity, despite surprisingly limited sequence specificity, is dependent on the size of the central region of PrP(C)...
  11. pmc Repetitive immunization enhances the susceptibility of mice to peripherally administered prions
    Juliane Bremer
    Institute of Neuropathology, Department of Pathology, University Hospital of Zurich, Zurich, Switzerland
    PLoS ONE 4:e7160. 2009
    ..Due to its profound impact onto scrapie susceptibility, the host immune status may determine disease penetrance after low-dose prion exposure, including those that may give rise to iatrogenic and variant Creutzfeldt-Jakob disease...
  12. pmc ZyFISH: a simple, rapid and reliable zygosity assay for transgenic mice
    Donal McHugh
    Department of Pathology, Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse, Switzerland
    PLoS ONE 7:e37881. 2012
    ..This combination of reliability, simplicity and cost-effectiveness makes zyFISH a method of choice for transgenic mouse zygosity determinations...
  13. pmc Preclinical deposition of pathological prion protein in muscle of experimentally infected primates
    Susanne Krasemann
    Institute of Neuropathology, University Medical Center Hamburg Eppendorf, Hamburg, Germany
    PLoS ONE 5:e13906. 2010
    ..Here, we show the preclinical presence of PrP(Sc) in muscle and central nervous system of rhesus monkeys experimentally infected with vCJD...
  14. pmc Ablation of Dicer from murine Schwann cells increases their proliferation while blocking myelination
    Juliane Bremer
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    PLoS ONE 5:e12450. 2010
    ..This set of microRNAs appears to drive Schwann cell differentiation and myelination of peripheral nerves, thereby fulfilling a crucial function for survival of the organism...
  15. pmc Prion pathogenesis is faithfully reproduced in cerebellar organotypic slice cultures
    Jeppe Falsig
    Institute of Neuropathology, Zurich, Switzerland
    PLoS Pathog 8:e1002985. 2012
    ....
  16. doi request reprint Follicular dendritic cells: origin, phenotype, and function in health and disease
    Adriano Aguzzi
    Institute of Neuropathology, University of Zurich, Zurich, Switzerland Electronic address
    Trends Immunol 35:105-13. 2014
    ..Here we review these findings, and discuss current concepts related to FDC histogenesis both in lymphoid organs and in inflammatory lymphoneogenesis. ..
  17. doi request reprint A template for new drugs against Alzheimer's disease
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital Zurich, 8091 Zurich, Switzerland Electronic address
    Cell 154:1182-4. 2013
    ..define a novel structural model of Aβ fibrils from AD brain, revealing surprising differences from in vitro fibrils. These findings may lead to structure-specific inhibitors and more selective amyloid-imaging methods...
  18. doi request reprint Microglia: scapegoat, saboteur, or something else?
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital Zurich, Schmelzbergstrasse 12, CH 8012 Zurich, Switzerland
    Science 339:156-61. 2013
    ..It also seems increasingly likely that microglial dysfunction can underlie certain neurological diseases without an obvious immune component...
  19. doi request reprint Prion propagation, toxicity and degradation
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
    Nat Neurosci 15:936-9. 2012
    ..Here we review these developments and summarize those open questions that we currently deem most interesting in prion biology: how do prions damage their hosts, and how do hosts attempt to neutralize invading prions?..
  20. pmc Cerebrospinal fluid biomarkers in human genetic transmissible spongiform encephalopathies
    Anna Ladogana
    Department of Cellular Biology and Neurosciences, Instituto Superiore di Sanita, Viale Regina Elena 299, 00161 Rome, Italy
    J Neurol 256:1620-8. 2009
    ..Disease duration and codon 129 genotype influence the findings in a different way than in sporadic CJD...
  21. pmc A case-control study of sporadic Creutzfeldt-Jakob disease in Switzerland: analysis of potential risk factors with regard to an increased CJD incidence in the years 2001-2004
    Jessica Ruegger
    Department of Neurology, University Hospital Zurich, Zurich, Switzerland
    BMC Public Health 9:18. 2009
    ..In 2001, the observed annual mortality from Creutzfeldt-Jakob disease (CJD) in Switzerland increased from less than 1.5 to 2.6 per million inhabitants. An underlying cause could not be identified...
  22. doi request reprint Characterizing follicular dendritic cells: A progress report
    Adriano Aguzzi
    Institute of Neuropathology, University of Zurich, Zurich, Switzerland
    Eur J Immunol 40:2134-8. 2010
    ..Here we review the current state of FDC science with specific reference to a study in this issue of the European Journal of Immunology and its efforts in discovering new FDC markers...
  23. ncbi request reprint Prion infections, blood and transfusions
    Adriano Aguzzi
    Department of Pathology, Zurich University, Switzerland
    Nat Clin Pract Neurol 2:321-9. 2006
    ..Novel diagnostic strategies, which might include the use of surrogate markers of prion infection, along with prion removal strategies, might help to control the risk of iatrogenic prion spread through blood transfusions...
  24. ncbi request reprint Insights into prion strains and neurotoxicity
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nat Rev Mol Cell Biol 8:552-61. 2007
    ..The existence of such strains poses a fascinating challenge to prion research...
  25. pmc Overexpression of lymphotoxin in T cells induces fulminant thymic involution
    Mathias Heikenwalder
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Am J Pathol 172:1555-70. 2008
    ....
  26. doi request reprint Protein aggregation diseases: pathogenicity and therapeutic perspectives
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH8091 Zurich, Switzerland
    Nat Rev Drug Discov 9:237-48. 2010
    ....
  27. ncbi request reprint Pathogenesis of prion diseases: current status and future outlook
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nat Rev Microbiol 4:765-75. 2006
    ..Here we focus on the unresolved issues pertaining to prion pathogenesis, particularly on the role played by the immune system...
  28. doi request reprint The transcellular spread of cytosolic amyloids, prions, and prionoids
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Neuron 64:783-90. 2009
    ....
  29. doi request reprint Prions: protein aggregation and infectious diseases
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    Physiol Rev 89:1105-52. 2009
    ..Finally, we discuss potential antiprion therapies and current developments in the realm of prion diagnostics...
  30. doi request reprint Prion topology and toxicity
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
    Cell 137:994-6. 2009
    ..Chakrabarti and Hegde (2009) now report that prion proteins with aberrant topologies inactivate mahogunin, providing a plausible explanation for certain aspects of prion pathology...
  31. pmc Progress and problems in the biology, diagnostics, and therapeutics of prion diseases
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
    J Clin Invest 114:153-60. 2004
    ..This article reviews recent progress in the areas of pathogenesis of, diagnostics of, and therapy for prion diseases and highlights some conspicuous problems that remain to be addressed in each of these fields...
  32. pmc Unraveling prion strains with cell biology and organic chemistry
    Adriano Aguzzi
    Institute of Neuropathology, Universitatsspital Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Proc Natl Acad Sci U S A 105:11-2. 2008
  33. doi request reprint Molecular mechanisms of prion pathogenesis
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, CH 8091 Zurich, Switzerland
    Annu Rev Pathol 3:11-40. 2008
    ..Here, we discuss the molecular mechanisms leading to neurodegeneration, the role of the immune system in prion pathogenesis, and the existence of prion strains that appear to have different tropisms and biochemical characteristics...
  34. pmc Follicular dendritic cells control engulfment of apoptotic bodies by secreting Mfge8
    Jan Kranich
    Institute of Neuropathology, University Hospital of Zurich, 8091 Zurich, Switzerland
    J Exp Med 205:1293-302. 2008
    ..Lymphotoxin-deficient mice lacked FDCs and splenic Mfge8, and suffer from autoimmunity similar to Mfge8(-/-) mice. Hence, FDCs facilitate TBMphi-mediated corpse removal, and their malfunction may be involved in autoimmunity...
  35. ncbi request reprint Transcriptional stability of cultured cells upon prion infection
    Christian Julius
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    J Mol Biol 375:1222-33. 2008
    ..We conclude that, surprisingly, there are no universal transcriptional changes induced by prion infection of neural cells in vitro...
  36. ncbi request reprint Truncated prion protein and Doppel are myelinotoxic in the absence of oligodendrocytic PrPC
    Ivan Radovanovic
    Institute of Neuropathology, University Hospital of Zurich, CH 8091 Zurich, Switzerland
    J Neurosci 25:4879-88. 2005
    ..These results identify white-matter damage as an extraneuronal PrP-associated pathology and suggest a previously unrecognized role of PrP(C) in myelin maintenance...
  37. ncbi request reprint Immune system and peripheral nerves in propagation of prions to CNS
    Adriano Aguzzi
    Institute of Neuropathology, Universitatsspital Zurich, Switzerland
    Br Med Bull 66:141-59. 2003
    ....
  38. doi request reprint Efficient amyloid A clearance in the absence of immunoglobulins and complement factors
    Jana Sponarova
    Institute of Neuropathology, University Hospital of Zurich, 8091 Zurich, Switzerland
    Am J Pathol 182:1297-307. 2013
    ..Hence, antibodies or complement factors are not necessary for natural amyloid clearance, implying the existence of alternative physiological pathways for amyloid removal...
  39. doi request reprint A lymphotoxin-driven pathway to hepatocellular carcinoma
    Johannes Haybaeck
    Department of Pathology, University Hospital Zurich, CH 8091 Zurich, Switzerland
    Cancer Cell 16:295-308. 2009
    ..Thus, sustained LT signaling represents a pathway involved in hepatitis-induced HCC...
  40. ncbi request reprint Early and rapid engraftment of bone marrow-derived microglia in scrapie
    Josef Priller
    Institute of Neuropathology, Department of Pathology, University of Zurich, 8091 Zurich, Switzerland
    J Neurosci 26:11753-62. 2006
    ..Hence, in contrast to previous reports, microglia are not competent for efficient prion transport and replication in vivo...
  41. ncbi request reprint Chronic lymphocytic inflammation specifies the organ tropism of prions
    Mathias Heikenwalder
    Institute of Neuropathology, University Hospital of Zurich, CH 8091 Zurich, Switzerland
    Science 307:1107-10. 2005
    ..By expanding the tissue distribution of prions, chronic inflammatory conditions may act as modifiers of natural and iatrogenic prion transmission...
  42. ncbi request reprint Intrinsic resistance of oligodendrocytes to prion infection
    Marco Prinz
    Institute of Neuropathology, University Hospital of Zurich, CH 8091 Zurich, Switzerland
    J Neurosci 24:5974-81. 2004
    ..Hence oligodendrocytes do not support cell-autonomous prion replication, establishment of subclinical disease, and neural spread of prions. Prion resistance sets oligodendrocytes aside from both neurons and astrocytes...
  43. pmc A versatile prion replication assay in organotypic brain slices
    Jeppe Falsig
    Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, Zurich, Switzerland
    Nat Neurosci 11:109-17. 2008
    ..This suggests that the extensive microglial activation accompanying prion diseases represents an efficacious defensive reaction...
  44. ncbi request reprint Germinal center B cells are dispensable in prion transport and neuroinvasion
    Mathias Heikenwalder
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    J Neuroimmunol 192:113-23. 2007
    ....
  45. doi request reprint Lymphotoxin-dependent prion replication in inflammatory stromal cells of granulomas
    Mathias Heikenwalder
    Institute of Neuropathology, Department of Pathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Immunity 29:998-1008. 2008
    ..Hence, granulomas can act as clinically silent reservoirs of prion infectivity. Furthermore, lymphotoxin-dependent prion replication can occur in inflammatory stromal cells that are distinct from FDCs...
  46. doi request reprint Axonal prion protein is required for peripheral myelin maintenance
    Juliane Bremer
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    Nat Neurosci 13:310-8. 2010
    ..These results indicate that neuronal expression and regulated proteolysis of PrP(C) are essential for myelin maintenance...
  47. doi request reprint The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein
    Tiziana Sonati
    Institute of Neuropathology, University Hospital Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nature 501:102-6. 2013
    ..Flexible tail-mediated toxicity may conceivably play a role in further prion pathologies, such as familial Creutzfeldt-Jakob disease in humans bearing supernumerary octapeptides. ..
  48. pmc De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis
    Christina J Sigurdson
    Universitatsspital Zurich, Institute of Neuropathology, CH 8091 Zurich, Switzerland
    Proc Natl Acad Sci U S A 106:304-9. 2009
    ..This shows that expression of a subtly altered prion protein, with known 3D structure, efficiently generates a prion disease...
  49. pmc Bacterial colitis increases susceptibility to oral prion disease
    Christina J Sigurdson
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    J Infect Dis 199:243-52. 2009
    ..Therefore, moderate intestinal inflammation at the time of prion exposure may constitute one of the elusive risk factors underlying the development of TSE...
  50. pmc Polythiophenes inhibit prion propagation by stabilizing prion protein (PrP) aggregates
    Ilan Margalith
    Institute of Neuropathology, University Hospital of Zurich, CH 8091 Zurich, Switzerland
    J Biol Chem 287:18872-87. 2012
    ..Therefore, LCPs represent a novel class of antiprion agents whose mode of action appears to rely on hyperstabilization, rather than destabilization, of PrP(Sc) deposits...
  51. doi request reprint Association between deposition of beta-amyloid and pathological prion protein in sporadic Creutzfeldt-Jakob disease
    Laura Debatin
    Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
    Neurodegener Dis 5:347-54. 2008
    ....
  52. pmc Lethal recessive myelin toxicity of prion protein lacking its central domain
    Frank Baumann
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    EMBO J 26:538-47. 2007
    ..These findings suggest that myelin integrity is maintained by a constitutively active neurotrophic protein complex involving PrP(C), whose effector domain encompasses residues 94-134...
  53. pmc Lymph nodal prion replication and neuroinvasion in mice devoid of follicular dendritic cells
    Marco Prinz
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Proc Natl Acad Sci U S A 99:919-24. 2002
    ..Macrophage subsets are plausible candidates for lymphoreticular prion pathogenesis and neuroinvasion in the absence of FDCs, and may represent a novel target for postexposure prophylaxis...
  54. pmc Follicular dendritic cells emerge from ubiquitous perivascular precursors
    Nike Julia Krautler
    Institute of Neuropathology, University Hospital of Zurich, 8091 Zurich, Switzerland
    Cell 150:194-206. 2012
    ..The ubiquity of preFDC and their strategic location at blood vessels may explain the de novo generation of organized lymphoid tissue at sites of lymphocytic inflammation...
  55. ncbi request reprint Lymphotoxin-beta receptor-dependent genes in lymph node and follicular dendritic cell transcriptomes
    Christoph Huber
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    J Immunol 174:5526-36. 2005
    ..Hence, the set of genes presented in this study includes markers emanating from LTbetaR signaling and transcripts relevant to GC and FDC function...
  56. ncbi request reprint Lymphoid follicle destruction and immunosuppression after repeated CpG oligodeoxynucleotide administration
    Mathias Heikenwalder
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nat Med 10:187-92. 2004
    ....
  57. pmc Disruption of Doppel prevents neurodegeneration in mice with extensive Prnp deletions
    Nicolas Genoud
    Institute of Neuropathology, University Hospital Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Proc Natl Acad Sci U S A 101:4198-203. 2004
    ..Alternatively, Dpl and PrP(C) may exert distinct functions despite having partly overlapping expression profiles...
  58. ncbi request reprint Expression of lymphotoxin beta governs immunity at two distinct levels
    Tobias Junt
    Institute of Experimental Immunology, Zurich, Switzerland
    Eur J Immunol 36:2061-75. 2006
    ..ii) Expression of LTbeta on non-lymphocytes governs antiviral immunity by enhancing antigen presentation on antigen-presenting cells. This prevents cytotoxic T lymphocytes exhaustion or death of the host by uncontrolled virus spread...
  59. ncbi request reprint Prion strain discrimination using luminescent conjugated polymers
    Christina J Sigurdson
    Universitatsspital Zurich, Institute of Neuropathology, Department of Pathology, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nat Methods 4:1023-30. 2007
    ..LCPs may help to detect structural differences among discrete protein aggregates and to link protein conformational features with disease phenotypes...
  60. ncbi request reprint Soluble dimeric prion protein binds PrP(Sc) in vivo and antagonizes prion disease
    Philipp Meier
    Institute of Neuropathology, Schmelzbergstrasse, University Hospital of Zurich, Zurich, Switzerland
    Cell 113:49-60. 2003
    ..The unique properties of PrP-Fc(2) suggest that soluble PrP derivatives may represent a new class of prion replication antagonists...
  61. ncbi request reprint Prion transmission prevented by modifying the β2-α2 loop structure of host PrPC
    Timothy D Kurt
    Departments of Pathology and Medicine, University of California, San Diego, La Jolla, California 92093, CIC bioGUNE, Parque Tecnologico de Bizkaia, 48160 Derio, Spain, Institut fur Molekularbiologie und Biophysik, ETH Zurich, CH 8093 Zurich, Switzerland, Institute of Laboratory Animal Science and Biomodels, University of Veterinary Medicine, 1210 Vienna, Austria, IKERBASQUE, Basque Foundation for Science, 48011 Bilbao, Spain, Department of Integrated Structural and Computational Biology, and Skaggs Institute for Chemical Biology, Scripps Research Institute, La Jolla, California 92037, Universitatsspital Zurich, Institute of Neuropathology, CH 8091 Zurich, Switzerland, and Department of Pathology, Immunology, and Microbiology, University of California, Davis, California 95616
    J Neurosci 34:1022-7. 2014
    ..These studies indicate that Y169 is important for prion formation, and they provide a strong indication that variation of the β2-α2 loop structure can modulate interspecies prion transmission. ..
  62. ncbi request reprint Stromal complement receptor CD21/35 facilitates lymphoid prion colonization and pathogenesis
    Mark D Zabel
    Institute for Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    J Immunol 179:6144-52. 2007
    ..Because both PrP(C) and CD21/35 are highly expressed on follicular dendritic cells, CD21/35 appears to be involved in targeting prions to follicular dendritic cells and expediting neuroinvasion following peripheral exposure to prions...
  63. pmc The POM monoclonals: a comprehensive set of antibodies to non-overlapping prion protein epitopes
    Magdalini Polymenidou
    Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
    PLoS ONE 3:e3872. 2008
    ....
  64. doi request reprint Heightened incidence of sporadic Creutzfeldt-Jakob disease is associated with a shift in clinicopathological profiles
    Katharina Stoeck
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland
    J Neurol 255:1464-72. 2008
    ....
  65. pmc Oral prion infection requires normal numbers of Peyer's patches but not of enteric lymphocytes
    Marco Prinz
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    Am J Pathol 162:1103-11. 2003
    ..Although susceptibility to prion infection following oral challenge correlates with the number of PPs, it is remarkably independent of the number of PP-associated lymphocytes...
  66. ncbi request reprint Coincident scrapie infection and nephritis lead to urinary prion excretion
    Harald Seeger
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Science 310:324-6. 2005
    ..Thus, urine may provide a vector for horizontal prion transmission, and inflammation of excretory organs may influence prion spread...
  67. ncbi request reprint Current concepts and controversies in prion immunopathology
    Mathias Heikenwalder
    Institute of Neuropathology, University Hospital of Zürich CH 8091 Zürich, Switzerland
    J Mol Neurosci 23:3-12. 2004
    ..M cells are a plausible candidate for the mucosal portal of prion infection...
  68. pmc Prion pathogenesis in the absence of Toll-like receptor signalling
    Marco Prinz
    University Hospital of Zurich, Zurich, Switzerland
    EMBO Rep 4:195-9. 2003
    ..TLR signalling therefore does not have any major role in prion pathogenesis, and the protective effect of TLR stimulation is unlikely to result from direct interactions with prions...
  69. ncbi request reprint Human prion diseases: molecular and clinical aspects
    Markus Glatzel
    Institute of Neuropathology and National Reference Center for Prion Diseases, University Hospital Zurich, Zurich, Switzerland
    Arch Neurol 62:545-52. 2005
    ..The present review article discusses current concepts and controversies surrounding the basic biological features of prions...
  70. pmc Lymphotoxin, but not TNF, is required for prion invasion of lymph nodes
    Tracy O'Connor
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    PLoS Pathog 8:e1002867. 2012
    ..Hence, prions may enter lymph nodes by HEVs and accumulate or replicate in the absence of mature FDCs...
  71. ncbi request reprint Experimental autoimmune encephalomyelitis repressed by microglial paralysis
    Frank L Heppner
    Institute of Neuropathology, University Hospital Zurich, CH 8091 Zurich, Switzerland
    Nat Med 11:146-52. 2005
    ..The microglial compartment thus provides a potential therapeutic target in inflammatory CNS disorders. These results validate CD11b-HSVTK mice as a tool to study the impact of microglial activation on CNS diseases in vivo...
  72. pmc Humoral immune response to native eukaryotic prion protein correlates with anti-prion protection
    Magdalini Polymenidou
    Institute of Neuropathology, Neuroimmunology Unit, University Hospital, Schmelzbergstrasse 12 and Frauenklinikstrasse 10, CH 8091 Zurich, Switzerland
    Proc Natl Acad Sci U S A 101:14670-6. 2004
    ..Its circumvention might represent an important step toward the development of effective anti-prion immunotherapy...
  73. doi request reprint Triggering TLR7 in mice induces immune activation and lymphoid system disruption, resembling HIV-mediated pathology
    Stefan Baenziger
    Division of Infectious Diseases and Hospital Epidemiology, University Hospital of Zurich, Zurich, Switzerland
    Blood 113:377-88. 2009
    ..Thus, manipulation of TLR7 signaling may be a potential strategy to reduce chronic hyper-immune activation and, thereby, disease progression in HIV infection...
  74. ncbi request reprint Reconstructing prions: fibril assembly from simple yeast to complex mammals
    Christina Sigurdson
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    Neurodegener Dis 2:1-5. 2005
    ..The simple yeast organism has served as a valuable model for understanding aspects of prion biology, such as prion fibril assembly...
  75. pmc Prion protein and Abeta-related synaptic toxicity impairment
    Anna Maria Calella
    Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
    EMBO Mol Med 2:306-14. 2010
    ..We report that ablation or overexpression of PrP(C) had no effect on the impairment of hippocampal synaptic plasticity in a transgenic model of AD. These findings challenge the role of PrP(C) as a mediator of Abeta toxicity...
  76. pmc Strain fidelity of chronic wasting disease upon murine adaptation
    Christina J Sigurdson
    Universitatsspital Zurich, Institute of Neuropathology, Department of Pathology, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    J Virol 80:12303-11. 2006
    ..These findings indicate that the salient strain-specific properties of CWD are encoded by agent-intrinsic components rather than by host factors...
  77. pmc Urinary alpha1-antichymotrypsin: a biomarker of prion infection
    Gino Miele
    Department of Pathology, Universitatsspital Zurich, Institute of Neuropathology, Zurich, Switzerland
    PLoS ONE 3:e3870. 2008
    ....
  78. ncbi request reprint The peripheral nervous system and the pathogenesis of prion diseases
    Markus Glatzel
    Institute of Neuropathology and National Reference Center for Prion Diseases, University Hospital Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Curr Mol Med 4:355-9. 2004
    ..In this article, we review the state of knowledge on the routes of neuroinvasion used by the infectious agent in order to gain access to the central nervous system upon entry into extracerebral sites...
  79. pmc Antiprion prophylaxis by gene transfer of a soluble prion antagonist
    Nicolas Genoud
    Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
    Am J Pathol 172:1287-96. 2008
    ..These results suggest that somatic gene transfer of prion antagonists may be effective for postexposure prophylaxis of prion diseases...
  80. doi request reprint Canine MDCK cell lines are refractory to infection with human and mouse prions
    Magdalini Polymenidou
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, Zurich, Switzerland
    Vaccine 26:2601-14. 2008
    ..These factors may account for their resistance to infection. Alternatively, prion resistance may be related to the specific sequence of canine Prnp, as suggested by the lack of documented prion diseases in dogs...
  81. ncbi request reprint No influence of amyloid-beta-degrading neprilysin activity on prion pathogenesis
    Markus Glatzel
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    J Gen Virol 86:1861-7. 2005
    ..Also, incubation times and clinical course of prion disease, as well as brain infectivity titres at terminal stage, were unaffected. These data rule out neprilysin as a major modulator of PrPSc accumulation and prion pathogenesis...
  82. ncbi request reprint vCJD tissue distribution and transmission by transfusion--a worst-case scenario coming true?
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, CH 8091 Zurich, Switzerland
    Lancet 363:411-2. 2004
  83. ncbi request reprint Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease
    Magdalini Polymenidou
    Institute of Neuropathology, University Hospital Zurich, Switzerland
    Lancet Neurol 4:805-14. 2005
    ..On digestion with proteinase K, type 1 and type 2 PrP(Sc) display unglycosylated core fragments of 21 kDa and 19 kDa, resulting from cleavage around amino acids 82 and 97, respectively...
  84. ncbi request reprint Unhampered prion neuroinvasion despite impaired fast axonal transport in transgenic mice overexpressing four-repeat tau
    Valérie Künzi
    Institute of Neuropathology, University Hospital Zurich, CH 8091 Zurich, Switzerland
    J Neurosci 22:7471-7. 2002
    ..The coincidence of tau and prion pathology resembled Gerstmann-Sträussler-Scheinker syndrome. These findings identify tau pathology as a possible end stretch of prion-induced neurodegeneration...
  85. ncbi request reprint Mammalian prion biology: one century of evolving concepts
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstr 12, CH 8091 Zurich, Switzerland
    Cell 116:313-27. 2004
    ..The two decades since Stanley Prusiner's formulation of the protein-only hypothesis have witnessed spectacular advances, and yet some of the most basic questions in prion science have remained unanswered...
  86. ncbi request reprint Cell biology. Prion toxicity: all sail and no anchor
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, CH 8091 Zurich, Switzerland
    Science 308:1420-1. 2005
  87. ncbi request reprint Recent developments in prion immunotherapy
    Frank L Heppner
    Institute of Neuropathology, University Hospital Zurich, Schmelzbergstrasse 12, CH 8091, Switzerland
    Curr Opin Immunol 16:594-8. 2004
    ..The difficulties involved in eliciting development of such anti-native PrPC immune responses may be partly intrinsic to B cells and, in addition, may reside in peripheral T helper tolerance...
  88. ncbi request reprint Variant Creutzfeldt-jakob disease: between lymphoid organs and brain
    Markus Glatzel
    Institute of Neuropathology, Zurich, Switzerland
    Trends Microbiol 12:51-3. 2004
    ..A recently published study demonstrated the presence of pathological prion protein in sympathetic ganglia of patients suffering from variant Creutzfeldt-Jakob disease, suggesting that these mechanisms might apply to humans...
  89. ncbi request reprint Antiprion immunotherapy: to suppress or to stimulate?
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital Zurich, Switzerland
    Nat Rev Immunol 4:725-36. 2004
    ..This article reviews the pathophysiology of prion diseases in mouse models and discusses their relevance to immunotherapeutic and immunoprophylactic antiprion strategies...
  90. ncbi request reprint No superoxide dismutase activity of cellular prion protein in vivo
    Gregor Hutter
    Institute of Neuropathology, Schmelzbergstrasse 12, University Hospital of Zurich, CH 8091 Zurich, Switzerland
    Biol Chem 384:1279-85. 2003
    ..We conclude that PrP(C) in vivo does not discernibly contribute to total SOD activity and does not possess an intrinsic dismutase activity...
  91. pmc Disseminated and sustained HIV infection in CD34+ cord blood cell-transplanted Rag2-/-gamma c-/- mice
    Stefan Baenziger
    Division of Infectious Diseases and Hospital Epidemiology, University Hospital Zurich, Raemistrasse 100, 8091 Zurich, Switzerland
    Proc Natl Acad Sci U S A 103:15951-6. 2006
    ....
  92. ncbi request reprint Prions--role of the peripheral nervous system
    Markus Glatzel
    Institute of Neuropathology, Zurich University Hospital, Switzerland
    Virus Res 82:53. 2002
  93. pmc Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia
    Eckhard Flechsig
    Institut fur Molekularbiologie, Universitat Zurich, CH 8057 Zurich, Switzerland
    EMBO J 22:3095-101. 2003
    ..Doppel and truncated PrP probably cause Purkinje cell degeneration by the same mechanism...
  94. ncbi request reprint Understanding the diversity of prions
    Adriano Aguzzi
    Nat Cell Biol 6:290-2. 2004
  95. ncbi request reprint Small is not beautiful: antagonizing functions for the prion protein PrP(C) and its homologue Dpl
    Axel Behrens
    Mammalian Genetics Laboratory, Imperial Cancer Research Fund, 44 Lincoln s Inn Fields, London, UK
    Trends Neurosci 25:150-4. 2002
    ..Although we are only beginning to understand its function, the discovery of Dpl has already provided some answers to long-standing questions and is transforming our understanding of prion biology...
  96. pmc Heat shock factor 1 regulates lifespan as distinct from disease onset in prion disease
    Andrew D Steele
    Whitehead Institute for Biomedical Research, Howard Hughes Medical Institute, Massachusetts Institute of Technology, Cambridge, MA 02142, USA
    Proc Natl Acad Sci U S A 105:13626-31. 2008
    ..Thus, HSF1 provides an important protective function that is specifically manifest after the onset of behavioral symptoms of prion disease...
  97. ncbi request reprint Recent advances in prion biology
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Curr Opin Neurol 17:337-42. 2004
    ..Finally, there are hardly any therapeutic strategies. However, not all is gloomy, and many recent developments have advanced our basic understanding of prion diseases...
  98. ncbi request reprint A pathogenic PrP mutation and doppel interfere with polarized sorting of the prion protein
    Armgard Uelhoff
    Adolf Butenandt Institute, Department of Biochemistry, Laboratory for Alzheimer s and Parkinson s Disease Research, Ludwig Maximilians University, 80336 Munich, Germany
    J Biol Chem 280:5137-40. 2005
    ..Furthermore, a pathogenic PrP missense mutation within the HD leads to aberrant apical sorting of PrP as well...
  99. ncbi request reprint Enteroglial and neuronal involvement without apparent neuron loss in ileal enteric nervous system plexuses from scrapie-affected sheep
    Giuseppe Marruchella
    Department of Comparative Biomedical Sciences, Faculty of Veterinary Medicine, University of Teramo, Teramo, Italy
    J Gen Virol 88:2899-904. 2007
    ..Furthermore, the infectious agent may be less pathogenic towards ENS neurons than it is towards central nervous system neurons...
  100. doi request reprint Fibrillar prion peptide PrP(106-126) treatment induces Dab1 phosphorylation and impairs APP processing and Abeta production in cortical neurons
    Rosalina Gavin
    Cellular and Molecular Basis of Neurodegeneration and Neurorepair, Department of Cell Biology, University of Barcelona, Spain
    Neurobiol Dis 30:243-54. 2008
    ..Taken together, these data indicate a link between intracellular events induced by exposure to extracellular fibrillar peptide or PrP(res), and APP processing and implicate Dab1 in this link...