Article
MSA prions exhibit remarkable stability and resistance to inactivation.
Acta neuropathologica - 1 Jan 2018
Woerman Amanda L, Kazmi Sabeen A, Patel Smita, Freyman Yevgeniy, Oehler Abby, Aoyagi Atsushi, Mordes Daniel A, Halliday Glenda M, Middleton Lefkos T, Gentleman Steve M, Olson Steven H, Prusiner Stanley B
Abstract excerpt
In multiple system atrophy (MSA), progressive neurodegeneration results from the protein α-synuclein misfolding into a self-templating prion conformation that spreads throughout the brain. MSA prions are transmissible to transgenic (Tg) mice expressing mutated human α-synuclein (TgM83+/-), inducing neurological disease following intracranial inoculation with brain homogenate from deceased patient samples. Noting...
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