Tau proteinopathies and the prion concept.

Abstract

The ordered assembly of a small number of proteins into amyloid filaments is central to age-related neurodegenerative diseases. Tau is the most commonly affected of these proteins. In sporadic diseases, assemblies of tau form in a stochastic manner in certain brain regions, from where they appear to spread in a deterministic way, giving rise to disease symptoms. Over the past decade, multiple lines of evidence have shown that assembled tau behaves like a prion. More recently, electron cryo-microscopy of tau filaments has shown that distinct conformers are present in different diseases, with no inter-individual variation for a given disease.