title: Neuroinflammation in prion diseases: concepts and targets for therapeutic intervention.
abstract: Prion infections of the central nervous system (CNS) are characterized by a reactive gliosis and the subsequent degeneration of neuronal tissue. The activation of glial cells, which precedes neuronal death, is likely to be initially caused by the deposition of misfolded, in part proteinase K-resistant, isoforms (termed PrP(TSE)) of the normal cellular prion protein (PrP(c)) in the brain. Proinflammatory cytokines and chemokines released by PrP(TSE)-activated glial cells and stressed neurons may contribute directly or indirectly to the disease development by enhancement and generalization of the gliosis and via cytotoxicity for neurons. Recent studies have illustrated that interfering with inflammatory responses may represent a therapeutic approach to slow down the course of disease development. Hence, a better understanding of driving factors in neuroinflammation may well contribute to the development of improved strategies for treatment of prion diseases.
authors.affiliation: Project Neurodegenerative Diseases, Robert-Koch-Institute, Nordufer 20, 13353 Berlin, Germany.
authors.forename: Constanze
authors.initials: C
authors.lastname: Riemer
authors.forename: Sandra
authors.initials: S
authors.lastname: Gültner
authors.forename: Ines
authors.initials: I
authors.lastname: Heise
authors.forename: Nikola
authors.initials: N
authors.lastname: Holtkamp
authors.forename: Michael
authors.initials: M
authors.lastname: Baier
mesh_terms: D001921:Brain / Q000378:metabolism / Q000473:pathology*; D018925:Chemokines / Q000378:metabolism; D016207:Cytokines / Q000378:metabolism; D007249:Inflammation / Q000378:metabolism / Q000473:pathology; D017096:Prion Diseases / Q000378:metabolism / Q000473:pathology*; D011328:Prions / Q000378:metabolism
chemical_list: D018925:Chemokines; D016207:Cytokines; D011328:Prions