Spongiform encephalopathies: Insights from transgenic models

• Published in: Advances in Virus Research Vol. 56; pp. 313 - 352
• Main Authors: Aguzzi, Adriano, Brandner, Sebastian, Fischer, Michael B., Furukawa, Hisako, Glatzel, Markus, Hawkins, Cynthia, Heppner, Frank L., Montrasio, Fabio, Navarro, Beatriz, Parizek, Petra, Pekarik, Vladimir, Prinz, Marco, Raeber, Alex J., Röckl, Christiane, Klein, Michael A.
• Format: Book Chapter Journal Article
• Language: English
• Published: United States Elsevier Science & Technology 2001
• Subjects: Animals; Central Nervous System - immunology; Central Nervous System - metabolism; Central Nervous System - physiopathology; Disease Models, Animal; Disease Susceptibility; Humans; Mice; Mice, Transgenic; Prion Diseases - genetics; Prion Diseases - immunology; Prion Diseases - metabolism; Prion Diseases - physiopathology; Prions - chemistry; Prions - classification; Prions - genetics; Prions - metabolism; Species Specificity; Structure-Activity Relationship
• ISBN: 9780120398560; 0120398567
• ISSN: 0065-3527; 1557-8399
• DOI: 10.1016/S0065-3527(01)56032-7

This chapter summarizes some of the transgenic mouse models that contributed to the current understanding of the pathogenesis of transmissible spongiform encephalopathies. Prion diseases, or transmissible spongiform encephalopathies, are neurological disorders caused by transmissible pathogens termed prions. Human prion diseases are characterized by extended incubation periods ranging from several months to decades that are followed by a progressive clinical phase presenting with severe dementia and ataxia. Clinical disease is always lethal: death can occur within as short a period as a few weeks but occasionally in a period of up to a few years. Peripheral prion pathogenesis, and ultimately neuroinvasion are dependent on the components of the host-immune system. Collectively, these processes require either B cells or their products. At least one B cell-dependent event is the acquisition of a functional follicular dendritic cell network within the germinal centers of peripheral lymphoid tissue. These cells are the major sites of extraneuronal prion protein (PrPc) expression and probably the principal sites of PrPSc accumulation.