Comparative studies in the A30P and A53T alpha-Synuclein C. elegans strains to investigate the molecular origins of Parkinson's Disease
Author(s)
Type
Journal Article
Abstract
The aggregation of α-synuclein is a hallmark of Parkinson's disease (PD) and a variety of related neurological disorders. A number of mutations in this protein, including A30P and A53T, are associated with familial forms of the disease. Patients carrying the A30P mutation typically exhibit a similar age of onset and symptoms as sporadic PD, while those carrying the A53T mutation generally have an earlier age of onset and an accelerated progression. We report two C. elegans models of PD (PDA30P and PDA53T), which express these mutational variants in the muscle cells, and probed their behavior relative to animals expressing the wild-type protein (PDWT). PDA30P worms showed a reduced speed of movement and an increased paralysis rate, control worms, but no change in the frequency of body bends. By contrast, in PDA53T worms both speed and frequency of body bends were significantly decreased, and paralysis rate was increased. α-Synuclein was also observed to be less well localized into aggregates in PDA30P worms compared to PDA53T and PDWT worms, and amyloid-like features were evident later in the life of the animals, despite comparable levels of expression of α-synuclein. Furthermore, squalamine, a natural product currently in clinical trials for treating symptomatic aspects of PD, was found to reduce significantly the aggregation of α-synuclein and its associated toxicity in PDA53T and PDWT worms, but had less marked effects in PDA30P. In addition, using an antibody that targets the N-terminal region of α-synuclein, we observed a suppression of toxicity in PDA30P, PDA53T and PDWT worms. These results illustrate the use of these two C. elegans models in fundamental and applied PD research.
Date Issued
2021-03-22
Date Acceptance
2021-02-16
Citation
Frontiers in Cell and Developmental Biology, 2021, 9, pp.1-10
ISSN
2296-634X
Publisher
Frontiers Media
Start Page
1
End Page
10
Journal / Book Title
Frontiers in Cell and Developmental Biology
Volume
9
Copyright Statement
© 2021 Perni, van der Goot, Limbocker, van Ham, Aprile, Xu, Flagmeier, Thijssen, Sormanni, Fusco, Chen, Challa, Kirkegaard, Laine, Ma, Müller, Sinnige, Kumita, Cohen, Seinstra, Kaminski Schierle, Kaminski, Barbut, De Simone, Knowles, Zasloff, Nollen, Vendruscolo and Dobson. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
License URL
Sponsor
Medical Research Council (MRC)
Alzheimer's Society
Identifier
http://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000636575500001&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=1ba7043ffcc86c417c072aa74d649202
Grant Number
MR/S033947/1
511
Subjects
Science & Technology
Life Sciences & Biomedicine
Cell Biology
Developmental Biology
C
elegans
Parkinson&apos
s disease
alpha-synuclein
drug discovery
protein aggregation
protein misfolding
neurodegenerative diseases
transgenic model
Publication Status
Published
Article Number
ARTN 552549
Date Publish Online
2021-03-22