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Cited by 282

The DOI matches its Crossref record; 97 of the 97 references it cites were found in a registry or an index.

Small-Molecule Modulation of TDP-43 Recruitment to Stress Granules Prevents Persistent TDP-43 Accumulation in ALS/FTD

Open access
Fang, Mark Y., Markmiller, Sebastian, Vu, Anthony Q., Javaherian, Ashkan, Dowdle, William E., Jolivet, Philippe, Bushway, Paul J., Castello, Nicholas A., Baral, Ashmita, Chan, Michelle Y., Linsley, Jeremy W., Linsley, Drew, Mercola, Mark, Finkbeiner, Steven, Lecuyer, Eric, Lewcock, Joseph W., Yeo, Gene W.·2019·Neuron

Abstract

Stress granules (SGs) form during cellular stress and are implicated in neurodegenerative diseases such as amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD). To yield insights into the role of SGs in pathophysiology, we performed a high-content screen to identify small molecules which alter SG properties in proliferative cells and human iPSC-derived motor neurons (iPS-MNs). One major class of active molecules contained extended planar aromatic moieties, suggesting a potential to intercalate in nucleic acids. Accordingly, we show that several hit compounds can prevent the RNA-dependent recruitment of the ALS-associated RNA-binding proteins (RBPs) TDP-43, FUS and HNRNPA2B1 into SGs. We further demonstrate that transient SG formation contributes to persistent accumulation of TDP-43 into cytoplasmic puncta and that our hit compounds can reduce this accumulation in iPS-MNs from ALS patients. We propose that compounds with planar moieties represent a promising starting point to develop small molecule therapeutics for treating ALS/FTD.

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Metadata

Authors
Fang, M. Y., Markmiller, S., Vu, A. Q., Javaherian, A., Dowdle, W. E., Jolivet, P., Bushway, P. J., Castello, N. A., Baral, A., Chan, M. Y., Linsley, J. W., Linsley, D., Mercola, M., Finkbeiner, S., Lecuyer, E., Lewcock, J. W., & Yeo, G. W.
Venue
Neuron
Publisher
Elsevier BV
Published
2019-09
Volume
103
Issue
5
Pages
802-819.e11
ISSN
0896-6273 (ISSN-L)
OpenAlex
W2954699006
Cited by
282
References
97
FWCI
16.86
Type
journal article
Language
en
Open access
bronze

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CoChat. (2026). Citation check: Small-Molecule Modulation of TDP-43 Recruitment to Stress Granules Prevents Persistent TDP-43 Accumulation in ALS/FTD. https://app.cochat.ai/papers/10.1016/j.neuron.2019.05.048
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Fang, M. Y., Markmiller, S., Vu, A. Q., Javaherian, A., Dowdle, W. E., Jolivet, P., Bushway, P. J., Castello, N. A., Baral, A., Chan, M. Y., Linsley, J. W., Linsley, D., Mercola, M., Finkbeiner, S., Lecuyer, E., Lewcock, J. W., & Yeo, G. W. (2019). Small-Molecule Modulation of TDP-43 Recruitment to Stress Granules Prevents Persistent TDP-43 Accumulation in ALS/FTD. Neuron. https://doi.org/10.1016/j.neuron.2019.05.048

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.