title: The Prion-Like Behavior of Assembled Tau in Transgenic Mice.
abstract: Tauopathies constitute neurodegenerative diseases that are characterized by the intracellular deposition of filaments made of hyperphosphorylated tau protein. The pattern of tau deposition in Alzheimer's disease follows a stereotypical progression, with the first lesions appearing in the locus coeruleus and entorhinal cortex, from where they appear to spread to the hippocampus and neocortex. Propagation of pathological tau is also characteristic of argyrophilic grain disease, where the lesions seem to spread through distinct regions of the limbic system. In chronic traumatic encephalopathy, tauopathy appears to spread from the neocortex to the brainstem. These findings implicate neuron-to-neuron propagation of tau aggregates. Isoform compositions and morphologies of tau filaments can differ between tauopathies, which is consistent with the existence of distinct tau strains. Here, we review recent findings that support prion-like mechanisms in the pathogenesis of tauopathies through the experimental use of transgenic mice.
authors.affiliation: Institute of Pathology, University Hospital, CH-4031 Basel, Switzerland.
authors.forename: Florence
authors.initials: F
authors.lastname: Clavaguera
authors.affiliation: Institute of Pathology, University Hospital, CH-4031 Basel, Switzerland.
authors.forename: Markus
authors.initials: M
authors.lastname: Tolnay
authors.affiliation: MRC Laboratory of Molecular Biology, Cambridge CB2 0QH, United Kingdom.
authors.forename: Michel
authors.initials: M
authors.lastname: Goedert
mesh_terms: D000544:Alzheimer Disease / Q000378:metabolism; D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D004195:Disease Models, Animal; D006801:Humans; D008125:Locus Coeruleus; D051379:Mice; D008822:Mice, Transgenic; D011328:Prions / Q000378:metabolism*; D024801:Tauopathies / Q000235:genetics / Q000378:metabolism*; D016875:tau Proteins / Q000235:genetics / Q000378:metabolism*
chemical_list: D011328:Prions; D016875:tau Proteins