Familial Parkinson's point mutation abolishes multiple system atrophy prion replication.
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ABSTRACT:
SUBMITTER: Woerman AL
PROVIDER: S-EPMC5777081 | biostudies-other | 2018 Jan
REPOSITORIES: biostudies-other
Publications
Familial Parkinson's point mutation abolishes multiple system atrophy prion replication.
Woerman Amanda L AL Kazmi Sabeen A SA Patel Smita S Aoyagi Atsushi A Oehler Abby A Widjaja Kartika K Mordes Daniel A DA Olson Steven H SH Prusiner Stanley B SB
Proceedings of the National Academy of Sciences of the United States of America 20171226 2
In the neurodegenerative disease multiple system atrophy (MSA), α-synuclein misfolds into a self-templating conformation to become a prion. To compare the biological activity of α-synuclein prions in MSA and Parkinson's disease (PD), we developed nine α-synuclein-YFP cell lines expressing point mutations responsible for inherited PD. MSA prions robustly infected wild-type, A30P, and A53T α-synuclein-YFP cells, but they were unable to replicate in cells expressing the E46K mutation. Coexpression ...[more]
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