Neurodegeneration and unfolded-protein response in mice expressing a membrane-tethered flexible tail of PrP.

The cellular prion protein (PrPC) consists of a flexible N-terminal tail (FT, aa 23-128) hinged to a membrane-anchored globular domain (GD, aa 129-231). Ligation of the GD with antibodies induces rapid neurodegeneration, which is prevented by deletion or functional inactivation of the FT. Therefore,...

Full description

Bibliographic Details
Main Authors: Paolo Dametto, Asvin K K Lakkaraju, Claire Bridel, Lukas Villiger, Tracy O'Connor, Uli S Herrmann, Pawel Pelczar, Thomas Rülicke, Donal McHugh, Arlind Adili, Adriano Aguzzi
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2015-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC4319788?pdf=render