title: The immunobiology of prion diseases.
abstract: Individuals infected with prions succumb to brain damage, and prion infections continue to be inexorably lethal. However, many crucial steps in prion pathogenesis occur in lymphatic organs and precede invasion of the central nervous system. In the past two decades, a great deal has been learnt concerning the cellular and molecular mechanisms of prion lymphoinvasion. These properties are diagnostically useful and have, for example, facilitated preclinical diagnosis of variant Creutzfeldt-Jakob disease in the tonsils. Moreover, the early colonization of lymphoid organs can be exploited for post-exposure prophylaxis of prion infections. As stromal cells of lymphoid organs are crucial for peripheral prion infection, the dedifferentiation of these cells offers a powerful means of hindering prion spread in infected individuals. In this Review, we discuss the current knowledge of the immunobiology of prions with an emphasis on how basic discoveries might enable translational strategies.
authors.affiliation: Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH-8091 Zurich, Switzerland.
authors.forename: Adriano
authors.initials: A
authors.lastname: Aguzzi
authors.forename: Mario
authors.initials: M
authors.lastname: Nuvolone
authors.forename: Caihong
authors.initials: C
authors.lastname: Zhu
mesh_terms: D000818:Animals; D002490:Central Nervous System / Q000473:pathology; D004195:Disease Models, Animal; D006801:Humans; D008221:Lymphoid Tissue / Q000276:immunology / Q000473:pathology*; D051379:Mice; D018621:PrPC Proteins / Q000276:immunology / Q000378:metabolism; D018620:PrPSc Proteins / Q000276:immunology / Q000378:metabolism; D017096:Prion Diseases / Q000276:immunology* / Q000635:transmission*; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics / Q000276:immunology; D021381:Protein Transport
chemical_list: C512656:PRNP protein, human; D018621:PrPC Proteins; D018620:PrPSc Proteins; D000072002:Prion Proteins; D011328:Prions