CNS pathogenesis of prion diseases.
Prion diseases or transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases, clinically characterised by cognitive decline, paralleled by severe damage to the central nervous system. Prion diseases have attracted a broad interest because of their unique mechanisms of replication and propagation; however, the underlying pathogenic mechanisms are still highly speculative. In this review, current knowledge about the pathogenesis of prion diseases in the CNS will be highlighted and the most revealing animal models will be discussed, with future perspectives to address immediate questions about the pathogenesis.
Brandner
Sebastian
S
MRC Prion Unit, Department of Neurodegenerative Diseases, Institute of Neurology, London, UK.
D000818:Animals; D016923:Cell Death; D002490:Central Nervous System / Q000378:metabolism* / Q000473:pathology; D004198:Disease Susceptibility; D006801:Humans; D051379:Mice; D023421:Models, Animal; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D018620:PrPSc Proteins / Q000378:metabolism; D017096:Prion Diseases / Q000209:etiology* / Q000378:metabolism* / Q000473:pathology; D011328:Prions / Q000378:metabolism*; D012608:Scrapie / Q000378:metabolism / Q000473:pathology; D012756:Sheep
D018620:PrPSc Proteins; D011328:Prions