A Aguzzi

Summary

Affiliation: University of Zurich
Country: Switzerland

Publications

  1. ncbi Prions: health scare and biological challenge
    A Aguzzi
    Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nat Rev Mol Cell Biol 2:118-26. 2001
  2. ncbi Pathogenesis of prion diseases: a progress report
    A Aguzzi
    Institute of Neuropathology, Department of Pathology, Schmelzbergstrasse 12, University Hospital Zurich, CH 8091 Zurich, Switzerland
    Cell Death Differ 7:889-902. 2000
  3. ncbi Prion diseases, blood and the immune system: concerns and reality
    A Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Haematologica 85:3-10. 2000
  4. ncbi Interventional strategies against prion diseases
    A Aguzzi
    Institute of Neuropathology, Universitatsspital Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nat Rev Neurosci 2:745-9. 2001
  5. ncbi Prion diseases
    A Aguzzi
    Department of Pathology, University of Zurich, Switzerland
    Haemophilia 4:619-27. 1998
  6. ncbi Antiprion immunotherapy: to suppress or to stimulate?
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital Zurich, Switzerland
    Nat Rev Immunol 4:725-36. 2004
  7. pmc Dangerous liaisons between a microbe and the prion protein
    Adriano Aguzzi
    Department of Pathology, University Hospital of Zurich, Schmelzbergstr 12, CH 8091 Zurich, Switzerland
    J Exp Med 198:1-4. 2003
  8. ncbi 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
  9. ncbi Prion diseases of humans and farm animals: epidemiology, genetics, and pathogenesis
    Adriano Aguzzi
    Institute of Neuropathology, Universitatsspital Zurich, Zurich, Switzerland
    J Neurochem 97:1726-39. 2006
  10. ncbi Prions and the immune system: a journey through gut, spleen, and nerves
    Adriano Aguzzi
    Institute of Neuropathology, Universitatsspital Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Adv Immunol 81:123-71. 2003

Research Grants

Detail Information

Publications133 found, 100 shown here

  1. ncbi Prions: health scare and biological challenge
    A Aguzzi
    Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nat Rev Mol Cell Biol 2:118-26. 2001
    ..What are the links between the bovine and human prion diseases, and how do prions reach and damage the central nervous system?..
  2. ncbi Pathogenesis of prion diseases: a progress report
    A Aguzzi
    Institute of Neuropathology, Department of Pathology, Schmelzbergstrasse 12, University Hospital Zurich, CH 8091 Zurich, Switzerland
    Cell Death Differ 7:889-902. 2000
    ..The latter developments have been astonishingly successful, and one could now argue that the prion principle is much more solidly established in yeast than in mammals...
  3. ncbi Prion diseases, blood and the immune system: concerns and reality
    A Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Haematologica 85:3-10. 2000
    ..This review article illustrates the problems with which research in this field is fraught, and presents some of the arguments which are controversially discussed in the field...
  4. ncbi Interventional strategies against prion diseases
    A Aguzzi
    Institute of Neuropathology, Universitatsspital Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nat Rev Neurosci 2:745-9. 2001
    ..Although it is unlikely that we will be able to cure the clinically overt stages of prion diseases in the foreseeable future, palliative and even life-prolonging interventions might no longer be confined to the realm of science fiction...
  5. ncbi Prion diseases
    A Aguzzi
    Department of Pathology, University of Zurich, Switzerland
    Haemophilia 4:619-27. 1998
    ..Precondition to achieving these goals is a thorough understanding of prion biology, and in particular of its protein chemistry...
  6. ncbi 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...
  7. pmc Dangerous liaisons between a microbe and the prion protein
    Adriano Aguzzi
    Department of Pathology, University Hospital of Zurich, Schmelzbergstr 12, CH 8091 Zurich, Switzerland
    J Exp Med 198:1-4. 2003
  8. ncbi 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...
  9. ncbi Prion diseases of humans and farm animals: epidemiology, genetics, and pathogenesis
    Adriano Aguzzi
    Institute of Neuropathology, Universitatsspital Zurich, Zurich, Switzerland
    J Neurochem 97:1726-39. 2006
    ..Biochemical and genetic evidence link PrP and its gene to the disease. Although TSEs are by definition transmissible, a growing number of Prnp-associated non-infectious neurodegenerative proteinopathies are now being recognized...
  10. ncbi Prions and the immune system: a journey through gut, spleen, and nerves
    Adriano Aguzzi
    Institute of Neuropathology, Universitatsspital Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Adv Immunol 81:123-71. 2003
    ..The investigation of these questions leads to unexpected detours, including the neurophysiology of lymphoid organs, and even the function of a prion protein homolog in male fertility...
  11. ncbi Prions, cytokines, and chemokines: a meeting in lymphoid organs
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Immunity 22:145-54. 2005
  12. ncbi Prions: pathogenesis and reverse genetics
    A Aguzzi
    Institute of Neuropathology, University Hospital Zurich, CH 8091 Zurich, Switzerland
    Ann N Y Acad Sci 920:140-57. 2000
    ..The latter studies helped in elucidating some of the mechanisms of prion spread and disease pathogenesis...
  13. ncbi 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
  14. ncbi 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
  15. ncbi 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...
  16. ncbi Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies
    F L Heppner
    Institute of Neuropathology, Institute of Laboratory Animal Science, Institute of Experimental Immunology, University Hospital Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Science 294:178-82. 2001
    ..Expression of endogenous PrP (PrP(C)) in the spleen and brain was unaffected, suggesting that immunity was responsible for protection. This indicates the feasibility of immunological inhibition of prion disease in vivo...
  17. ncbi Neuroinvasion of prions: insights from mouse models
    S Brandner
    Institute of Neuropathology, Department of Pathology, University Hospital, Zurich, Switzerland
    Exp Physiol 85:705-12. 2000
    ..The latter studies have allowed us to clarify some of the mechanisms of prion spread and disease pathogenesis...
  18. pmc Early induction of angiogenetic signals in gliomas of GFAP-v-src transgenic mice
    J P Theurillat
    Institute of Neuropathology, Department of Pathology, Universitatsspital Zurich, Switzerland
    Am J Pathol 154:581-90. 1999
    ..Given the close similarities in the progression pattern between GFAP-v-src and human gliomas, the present results suggest that these mice may provide a useful tool for antiangiogenic therapy research...
  19. pmc B lymphocyte-restricted expression of prion protein does not enable prion replication in prion protein knockout mice
    F Montrasio
    Institute of Molecular Biology, University of Zurich, Winterthurerstrasse 190, CH 8057 Zurich, Switzerland
    Proc Natl Acad Sci U S A 98:4034-7. 2001
    ..We conclude that splenic B lymphocytes are not prion-replication competent and that they acquire prions from other cells, most likely follicular dendritic cells with which they closely associate and whose maturation depends on them...
  20. ncbi Similar turnover and shedding of the cellular prion protein in primary lymphoid and neuronal cells
    P Parizek
    Institute of Neuropathology, Schmelzbergstr. 12, University Hospital, , Switzerland
    J Biol Chem 276:44627-32. 2001
    ..Soluble PrP was detected in murine and human serum, suggesting that it might be of physiological relevance...
  21. ncbi Sympathetic innervation of lymphoreticular organs is rate limiting for prion neuroinvasion
    M Glatzel
    Institute of Neuropathology, University Hospital Zurich, Schmelzbergstrasse 12, CH-8091, Zurich, Switzerland
    Neuron 31:25-34. 2001
    ..We conclude that sympathetic innervation of lymphoid organs is rate limiting for prion neuroinvasion and that splenic sympathetic nerves may act as extracerebral prion reservoirs...
  22. pmc Normal host prion protein (PrPC) is required for scrapie spread within the central nervous system
    S Brandner
    Institute of Neuropathology, University Hospital, Zurich, Switzerland
    Proc Natl Acad Sci U S A 93:13148-51. 1996
    ..Again, intraocular inoculation did not lead to disease in the PrP-producing graft. These results demonstrate that PrP is necessary for prion spread along neural pathways...
  23. ncbi Transgenic and knockout mice in research on prion diseases
    A J Raeber
    Institute of Neuropathology, Department of Pathology, University Hospital, Zurich, Switzerland
    Brain Pathol 8:715-33. 1998
    ..Such studies have shed new light onto the mechanisms of prion spread and disease pathogenesis...
  24. ncbi [Immunity against prions?]
    F L Heppner
    Institut für Neuropathologie, Universitatsspital Zurich, Switzerland
    Dtsch Med Wochenschr 127:328-30. 2002
  25. ncbi Complement facilitates early prion pathogenesis
    M A Klein
    Institute of Neuropathology, University of Zurich, Schmelzbergstrasse, Zurich, Switzerland
    Nat Med 7:488-92. 2001
    ..Splenic accumulation of prion infectivity and PrPSc was delayed, indicating that activation of specific complement components is involved in the initial trapping of prions in lymphoreticular organs early after infection...
  26. ncbi Overexpression of Pax5 is not sufficient for neoplastic transformation of mouse neuroectoderm
    J P Steinbach
    Institute of Neuropathology, University Hospital, Zurich, Switzerland
    Int J Cancer 93:459-67. 2001
    ..These results strongly argue against a dominant transforming activity of PAX5 in NEC and in cerebellar granule cell precursors of mice, and underline the restricted tissue-specificity of PAX5 related oncogenesis...
  27. ncbi No complementation between TP53 or RB-1 and v-src in astrocytomas of GFAP-v-src transgenic mice
    A S Maddalena
    Institute of Neuropathology, University Hospital of Zurich, Switzerland
    Brain Pathol 9:627-37. 1999
    ....
  28. ncbi Insertional mutagenesis of preneoplastic astrocytes by Moloney murine leukemia virus
    T A Afanasieva
    Institute of Neuropathology, University of Zurich, Zurich, Switzerland
    J Neurovirol 7:169-81. 2001
    ..We expect this method to be useful for identifying genes involved in astrocytoma development/progression in animal models...
  29. pmc Studies on prion replication in spleen
    A J Raeber
    Institute of Neuropathology, University Hospital, Zurich, Switzerland
    Dev Immunol 8:291-304. 2001
    ..These results imply that splenic lymphocytes can acquire prions, possibly from FDC's, but only if they express PrP...
  30. pmc Efficient lymphoreticular prion propagation requires PrP(c) in stromal and hematopoietic cells
    P S Kaeser
    Institute of Neuropathology, University Hospital, 8091 Zurich, Switzerland
    J Virol 75:7097-106. 2001
    ....
  31. ncbi Prion proteins and infertility: insight from mouse models
    N Genoud
    Institute of Neuropathology, Universitatsspital Zurich, Zurich, Switzerland
    Cytogenet Genome Res 103:285-9. 2003
    ..This phenotype shifts the attention from the brain, where most of the investigations on Dpl have been performed, to testis, raising hope to resolve the long lasting search of PrP(C) function...
  32. ncbi The heavy metal-responsive transcription factor-1 (MTF-1) is not required for neural differentiation
    P Lichtlen
    Institute of Molecular Biology, University of Zurich, Switzerland
    Biol Chem 380:711-5. 1999
    ..Such transplants devoid of MTF-1 may provide a useful tool for the further investigation of the effect of cell stress, including oxidative stress...
  33. ncbi Reduced latency but no increased brain tumor penetrance in mice with astrocyte specific expression of a human p53 mutant
    M A Klein
    Institute of Neuropathology, University Hospital of Zurich, Switzerland
    Oncogene 19:5329-37. 2000
    ..This reflects well observations from human astrocytic neoplasms with p53 mutations...
  34. pmc Astrocyte-specific expression of hamster prion protein (PrP) renders PrP knockout mice susceptible to hamster scrapie
    A J Raeber
    Institut fur Molekularbiologie, Abteilung I, University of Zurich, H onggerberg, 8093 Z urich, Switzerland
    EMBO J 16:6057-65. 1997
    ..Interestingly, mice expressing the same transgenes but also endogenous murine PrP genes propagated infectivity without developing disease...
  35. ncbi Deletions in the spinal muscular atrophy gene region in a newborn with neuropathy and extreme generalized muscular weakness
    M Hergersberg
    Institute of Medical Genetics, University of Zurich, Switzerland
    Eur J Paediatr Neurol 4:35-8. 2000
    ..This observation illustrates that severe congenital neuropathy can result from deletions in the SMN gene...
  36. ncbi Distal axonopathy in peripheral nerves of PMP22-mutant mice
    S Sancho
    Department of Cell Biology, Swiss Federal Institute of Technology and Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    Brain 122:1563-77. 1999
    ....
  37. ncbi 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. ncbi Sleep-wake disturbances in sporadic Creutzfeldt-Jakob disease
    H P Landolt
    Institute of Pharmacology and Toxicology, University of Zurich, Zurich, Switzerland
    Neurology 66:1418-24. 2006
    ..The prevalence and characteristics of sleep-wake disturbances in sporadic Creutzfeldt-Jakob disease (sCJD) are poorly understood...
  39. pmc Normal neurogenesis and scrapie pathogenesis in neural grafts lacking the prion protein homologue Doppel
    A Behrens
    Institute of Neuropathology, Universitatsspital Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    EMBO Rep 2:347-52. 2001
    ..Therefore, Dpl is unlikely to exert a cell-autonomous function during neural differentiation and, in contrast to its homologue PrPC, is dispensable for prion disease progression and for generation of PrPSc...
  40. ncbi The shifting biology of prions
    M Glatzel
    Department of Pathology, Institute of Neuropathology, Schmelzbergstrasse 12, University Hospital Zurich, CH-8091, Zurich, Switzerland
    Brain Res Brain Res Rev 36:241-8. 2001
    ..Despite their rarity, prion diseases have become an important topic in public health and basic research because of the connection between nvCJD and BSE and also because of the unusual biological attributes of the infectious agent...
  41. ncbi A crucial role for B cells in neuroinvasive scrapie
    M A Klein
    Institute of Neuropathology, University of Zurich, Switzerland
    Nature 390:687-90. 1997
    ..We conclude that differentiated B cells are crucial for neuroinvasion by scrapie, regardless of the specificity of their receptors...
  42. ncbi Bovine spongiform encephalopathy and early onset variant Creutzfeldt-Jakob disease
    C Weissmann
    Institut für Molekularbiologie I, Universitat Zurich, Zurich, Switzerland
    Curr Opin Neurobiol 7:695-700. 1997
    ..Recent evidence links the appearance of new variant Creutzfeldt-Jakob disease in humans to consumption of BSE-contaminated cattle-derived products...
  43. ncbi Binding of disease-associated prion protein to plasminogen
    M B Fischer
    Institute for Neuropatholgy, University Hospital of Zurich, Switzerland
    Nature 408:479-83. 2000
    ..Therefore, plasminogen represents the first endogenous factor discriminating between normal and pathological prion protein. This unexpected property may be exploited for diagnostic purposes...
  44. ncbi PrP(C) expression in the peripheral nervous system is a determinant of prion neuroinvasion
    M Glatzel
    Institute of Neuropathology, University Hospital Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    J Gen Virol 81:2813-21. 2000
    ..0.7 mm per day, and prion infectivity titres of sciatic nerves were much higher in tga20 than in wild-type mice, suggesting that overexpression of PrP(C) modulates the capacity for intranerval transport...
  45. ncbi 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...
  46. doi 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
    ....
  47. ncbi 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...
  48. pmc Embryonic lethality and liver degeneration in mice lacking the metal-responsive transcriptional activator MTF-1
    C Gunes
    Institut fur Molekularbiologie, Abteilung II der Universität Zürich, Zurich, Switzerland
    EMBO J 17:2846-54. 1998
    ..We also note that the MTF-1 null mutant phenotype bears some similarity to those of two other regulators of cellular stress response, namely c-Jun and NF-kappaB (p65/RelA)...
  49. doi Prion propagation in mice lacking central nervous system NF-kappaB signalling
    C Julius
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    J Gen Virol 89:1545-50. 2008
    ..In contrast to previously published data, the observed results showed no evidence supporting the hypothesis that impaired NF-kappaB signalling in the CNS impacts on prion pathogenesis...
  50. ncbi Normal host prion protein necessary for scrapie-induced neurotoxicity
    S Brandner
    Department of Pathology, University Hospital, Zurich, Switzerland
    Nature 379:339-43. 1996
    ..Therefore, in addition to being resistant to scrapie infection, brain tissue devoid of PrPC is not damaged by exogenous PrPSc...
  51. ncbi 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...
  52. 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...
  53. ncbi Sympathetic prions
    M Glatzel
    Institute of Neuropathology, University Hospital Zurich
    ScientificWorldJournal 1:555-6. 2001
    ....
  54. pmc Human MxA protein protects mice lacking a functional alpha/beta interferon system against La crosse virus and other lethal viral infections
    H P Hefti
    Institute of Medical Virology, University of Zurich, Zurich, Switzerland
    J Virol 73:6984-91. 1999
    ..Moreover, the results suggest that MxA may protect humans from potential fatal infections by La Crosse virus and other viral pathogens...
  55. ncbi Hypersensitivity to seizures in beta-amyloid precursor protein deficient mice
    J P Steinbach
    Institute of Neuropathology, University Hospital, Zurich, Switzerland
    Cell Death Differ 5:858-66. 1998
    ..Since enhanced seizures were observed also in mice homozygous for a deletion of the entire beta-APP gene, this phenotype results from a loss of APP rather than from a dominant effect of APPDelta...
  56. ncbi [Significance of prion protein in transmission of prions and in pathogenesis of spongiform encephalopathies]
    A J Raeber
    Institut für Neuropathologie, Universitatsspital, Zurich, Schweiz
    Wien Med Wochenschr 148:74-7. 1998
    ..In addition, B lymphocytes have been found to play a crucial role in the neuroinvasiveness of prions...
  57. ncbi Resuscitative hypothermia protects the neonatal rat brain from hypoxic-ischemic injury
    J Nedelcu
    Department of Magnetic Resonance, University Children s Hospital Zurich, Switzerland
    Brain Pathol 10:61-71. 2000
    ..Even then, the time to develop infarction was significantly prolonged, leaving the opportunity for additional therapeutic intervention...
  58. ncbi 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
    ....
  59. ncbi 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...
  60. ncbi 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...
  61. ncbi 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...
  62. ncbi The prion gene is associated with human long-term memory
    Andreas Papassotiropoulos
    Division of Psychiatry Research, University of Zurich, 8032 Zurich, Switzerland
    Hum Mol Genet 14:2241-6. 2005
    ..These results suggest a role for the prion protein in the formation of long-term memory in humans...
  63. ncbi 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...
  64. doi 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...
  65. 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...
  66. ncbi Human Toll-like receptor 2 mediates induction of the antimicrobial peptide human beta-defensin 2 in response to bacterial lipoprotein
    T Birchler
    Division of Immunology, Hematology, Oncology, Infectiology, Zurich University Children's Hospital, Zurich, Switzerland
    Eur J Immunol 31:3131-7. 2001
    ..Thus, exposure of human cells to bacterial lipoprotein elicits production of the antimicrobial peptide beta-defensin 2 through Toll-like receptor 2...
  67. pmc Measles virus spread and pathogenesis in genetically modified mice
    B Mrkic
    Institut für Molekularbiologie Abt I, Universitat Zurich, Zurich, Switzerland
    J Virol 72:7420-7. 1998
    ..We conclude that in mice alpha/beta interferon controls MV infection and that a high-affinity receptor facilitates, but is not strictly required for, MV spread and pathogenesis...
  68. 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)...
  69. ncbi 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...
  70. ncbi 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
    ....
  71. doi Cells and prions: a license to replicate
    Mario Nuvolone
    Department of Pathology, Institutes of Neuropathology, University Hospital Zurich, Switzerland
    FEBS Lett 583:2674-84. 2009
    ..However, recent data point to a broader range of cell types that can replicate prions. Here, we review the state of the art in regards to peripheral prion replication, neuroinvasion and the determinants of prion replication competence...
  72. doi 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
    ....
  73. doi 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...
  74. 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...
  75. 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...
  76. doi 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...
  77. doi 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
    ....
  78. doi 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...
  79. 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
    ....
  80. ncbi 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...
  81. 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...
  82. doi 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...
  83. ncbi 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...
  84. ncbi Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease
    Markus Glatzel
    Institute of Neuropathology and National Reference Center for Prion Diseases, University Hospital of Zurich, Zurich, Switzerland
    N Engl J Med 349:1812-20. 2003
    ..Understanding the distribution of PrPSc in Creutzfeldt-Jakob disease is important for classification and diagnosis and perhaps even for prevention...
  85. ncbi Analysis of the prion protein in primates reveals a new polymorphism in codon 226 (Y226F)
    Markus Glatzel
    Institute of Neuropathology, University Hospital Zurich, Switzerland
    Biol Chem 383:1021-5. 2002
    ..A modulatory effect of this polymorphism on the development of prion disease is possible because codon 226 is close to the suggested binding side of the factor X, which has been invoked as a determinant of the prion species barrier...
  86. ncbi 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...
  87. 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...
  88. 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...
  89. 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...
  90. ncbi 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...
  91. ncbi Cellular and molecular aspects of neurocarcinogenesis
    P Kleihues
    Department of Pathology, , Switzerland
    Toxicol Pathol 18:193-203. 1990
    ..Following infection of fetal donor cells with a vector encoding the v-myc oncogene, only a single embryonal CNS tumor was observed whereas exposure to v-H-ras produced a low incidence of gliomas.(ABSTRACT TRUNCATED AT 400 WORDS)..
  92. ncbi Development and malignant progression of astrocytomas in GFAP-v-src transgenic mice
    J Weissenberger
    Institute of Neuropathology, University Hospital of Zurich, Switzerland
    Oncogene 14:2005-13. 1997
    ..Time course and stochastic nature of the process indicate that v-src did not suffice for malignant transformation, and that astrocytomas were the result of a multistep process necessitating co-operation of additional genetic events...
  93. ncbi Expression of the interferon-induced MxA protein in viral encephalitis
    J B Lampe
    Institute of Neuropathology, Department of Pathology, University Hospital of Zurich, Schmelzbergstrasse 12, Zurich, Switzerland
    Neuropathol Appl Neurobiol 29:273-9. 2003
    ..Therefore, the detection of MxA-protein is a sensitive adjuvant marker for those cases of viral encephalitis which are accompanied by pronounced lymphocytic infiltrates...
  94. pmc Benzodiazepine-insensitive mice generated by targeted disruption of the gamma 2 subunit gene of gamma-aminobutyric acid type A receptors
    U Gunther
    Institute of Pharmacology, University of Zurich, Switzerland
    Proc Natl Acad Sci U S A 92:7749-53. 1995
    ..The lack of postnatal GABAA receptor regulation by endogenous ligands of BZ sites might contribute to this phenotype...
  95. ncbi 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...
  96. ncbi 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...
  97. 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
  98. 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...
  99. 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...
  100. ncbi Prions--role of the peripheral nervous system
    Markus Glatzel
    Institute of Neuropathology, Zurich University Hospital, Switzerland
    Virus Res 82:53. 2002
  101. 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...

Research Grants1

  1. Conference on Transmissible Spongiform Encephalopathies
    Adriano Aguzzi; Fiscal Year: 2005
    ....