Prion Diseases

1. Front Matter

   Pages i-ix

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2. Pathogenesis of Prion Diseases

   1. Front Matter

      Pages 1-1

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   2. Prions: from neurografts to neuroinvasion

      • M. Glatzel, M. A. Klein, S. Brandner, A. Aguzzi

      Pages 3-12

   3. Follicular dendritic cells in scrapie pathogenesis

      • K. L. Brown, K. Stewart, D. Ritchie, H. Fraser, W. I. Morrison, M. E. Bruce

      Pages 13-21

   4. Cellular and sub-cellular localisation of PrP in the lymphoreticular system of mice and sheep

      • M. Jeffrey, G. McGovern, S. Martin, C. M. Goodsir, K. L. Brown

      Pages 23-38

   5. Pharmacological manipulation of early PrPres accumulation in the spleen of scrapie-infected mice

      • V. Beringue, F. Lamoury, K. T. Adjou, T. Maignien, M. Demoy, P. Couvreur et al.

      Pages 39-56

   6. Pathogenesis of natural scrapie in sheep

      • L. J. M. van Keulen, B. E. C. Schreuder, M. E. W. Vromans, J. P. M. Langeveld, M. A. Smits

      Pages 57-71

3. Animal Models for Prion Diseases

   1. Front Matter

      Pages 73-73

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   2. Detection of cattle-derived BSE prions using transgenic mice overexpressing bovine PrPc

      • A. Buschmann, E. Pfaff, K. Reifenberg, H. M. Müller, M. H. Groschup

      Pages 75-86

   3. Analyzing the influence of PrP primary structure on prion pathogenesis in transgenic mice

      • S. Campbell, U. Dennehy, G. Telling

      Pages 87-94

   4. A single amino acid alteration in murine PrP dramatically alters TSE incubation time

      • J. C. Manson, R. Barron, E. Jamieson, H. Baybutt, N. Tuzi, I. McConnell et al.

      Pages 95-102

   5. A transgenic model of a familial prion disease

      • D. A. Harris, R. Chiesa, B. Drisaldi, E. Quaglio, A. Migheli, P. Piccardo et al.

      Pages 103-112

   6. Transgenic models of prion disease

      • M. R. Scott, S. Supattapone, H.-O. B. Nguyen, S. J. DeArmond, S. B. Prusiner

      Pages 113-124

4. Epidemiology and Diagnosis of Prion Diseases

   1. Front Matter

      Pages 125-125

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   2. Surveillance of BSE

      • D. Heim, J. W. Wilesmith

      Pages 127-133

   3. Histopathology and immunohistochemistry of human transmissible spongiform encephalopathies (TSEs)

      • H. Budka

      Pages 135-142

   4. Pathology of variant Creutzfeldt-Jakob disease

      • J. W. Ironside

      Pages 143-151

   5. Clinical and differential diagnosis of Creutzfeldt-Jakob disease

      • S. Poser, I. Zerr, A. Schroeter, M. Otto, A. Giese, B. J. Steinhoff et al.

      Pages 153-159

   6. Putting prions into focus: application of single molecule detection to the diagnosis of prion diseases

      • A. Giese, J. Bieschke, M. Eigen, H. A. Kretzschmar

      Pages 161-171

   7. Detection of PrPSc in subclinical BSE with the paraffin-embedded tissue (PET) blot

      • W. J. Schulz-Schaeffer, R. Fatzer, M. Vandevelde, H. A. Kretzschmar

      Pages 173-180

A comprehensive understanding of the biology of prion diseases is crucial for risk assessment as regards both humans and animals. To further the communication of the current knowledge and the exchange of diagnostic technologies in the scientific community, a symposium on the 'Characterization and Diagnosis of Prion Diseases' was held from September 23-25, 1999 in Tübingen, Germany. The overwhelming interest and the participation of more than 500 scientists from 26 countries made this symposium the largest meeting ever held in the field. In this Supplementum to Archives of Virology twenty-six invited speakers comprehensively present their data on the pathogenesis of prion diseases in humans and animals, on molecular mechanisms involved in the transmissibility across species barriers, on animal and in-vitro models currently available for the detection and quantification of infectivity and on the characterization of prion strains. This selection attempts to reflect the current state of the art, but cannot possibly represent the whole spectrum of research in the prion field.

• BSE
• Creutzfeld-Jakob Krankheit
• Creutzfeldt-Jakob disease
• PET
• Prionen
• antibody
• bovine spongiform encephalopathy
• bovine spongioforme Enzephalopathie
• dendritic cells
• differential diagnosis
• pathogenesis
• positron emission tomography (PET)
• prions
• tissue
• virology

• Institut für Immunologie, Bundesforschungsanstalt für Viruskrankheiten der Tiere, Tübingen, Germany

  Martin H. Groschup

• Institut für Neuropathologie, Ludwig-Maximilians-Universität, München, Germany

  Hans A. Kretzschmar

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