Article
The E46K mutation modulates α-synuclein prion replication in transgenic mice.
PLoS pathogens - 1 Dec 2022
Holec Sara A M, Lee Jisoo, Oehler Abby, Batia Lyn, Wiggins-Gamble Aryanna, Lau Jeffrey, Ooi Felicia K, Merz Gregory E, Wang Man, Mordes Daniel A, Olson Steven H, Woerman Amanda L
Abstract excerpt
In multiple system atrophy (MSA), the α-synuclein protein misfolds into a self-templating prion conformation that spreads throughout the brain, leading to progressive neurodegeneration. While the E46K mutation in α-synuclein causes familial Parkinson's disease (PD), we previously discovered that this mutation blocks in vitro propagation of MSA prions. Recent studies by others indicate that α-synuclein adopts a...
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