Striatal neuron dysfunction in C9ORF72-FTD/ALS is driven by AIS and potassium channel dysregulation.
Overview
- Sheffield Institute for Translational Neuroscience, Division of Neuroscience, University of Sheffield, Sheffield S10 2HQ, UK
- The Neuroscience Institute, University of Sheffield, Sheffield S10 2HQ, UK
Abstract
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) form a neurodegenerative spectrum characterized by progressive cognitive, behavioral, and motor decline, yet the contribution of the striatum to disease pathophysiology remains poorly understood. Here, we generate inhibitory striatal medium spiny neurons (MSNs) from human induced pluripotent stem cells carrying the C9ORF72 repeat expansion, the most common genetic cause of FTD/
Reproduced under the paper's license (CC BY), from the paper cited above.
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• All data reported in this paper will be shared by the lead contact upon request. • This paper does not report original code. • Any additional information required to reanalyze the data reported in this paper is available from the lead contact upon request.
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Versions
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Version 2, 28 September 2026
- Publisher: — → Cell Press
Version 1, 27 September 2026: the first record
Recorded: type, language, journal, volume, issue, pages, dates, 10 authors, 9 keywords, 11 MeSH terms, 3 funders, 50 references.
Cite
This paper
Pasniceanu, I.-S., Atwal, M. S., Santos Souza, C. D., Moll, T., King, M., Treanor, C., Cabezas de la Fuente, D., West, R. J., Ferraiuolo, L., & Livesey, M. R. (2026). Striatal neuron dysfunction in C9ORF72-FTD/
BibTeX
@article{pasniceanu2026s
author = {Pasniceanu, Iris-Stefania and Atwal, Manpreet S. and Santos Souza, Cleide Dos and Moll, Tobias and King, Marianne and Treanor, Connie and Cabezas de la Fuente, Daniel and West, Ryan J.H. and Ferraiuolo, Laura and Livesey, Matthew R.},
title = {{Striatal neuron dysfunction in C9ORF72-FTD/
journal = {Cell reports},
year = {2026},
month = jul,
volume = {45},
number = {7},
pages = {117672},
publisher = {Cell Press},
issn = {2211-1247},
doi = {10.1016/
url = {https://
pmid = {42412610},
pmcid = {PMC13415681}
}
RIS
TY - JOUR
AU - Pasniceanu, Iris-Stefania
AU - Atwal, Manpreet S.
AU - Santos Souza, Cleide Dos
AU - Moll, Tobias
AU - King, Marianne
AU - Treanor, Connie
AU - Cabezas de la Fuente, Daniel
AU - West, Ryan J.H.
AU - Ferraiuolo, Laura
AU - Livesey, Matthew R.
TI - Striatal neuron dysfunction in C9ORF72-FTD/
T2 - Cell reports
J2 - Cell Rep
PY - 2026
DA - 2026/
VL - 45
IS - 7
SP - 117672
SN - 2211-1247
PB - Cell Press
DO - 10.1016/
UR - https://
LA - en
ER -
CSL-JSON
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