Homozygous ALS-linked mutations in TARDBP/TDP-43 lead to hypoactivity and synaptic abnormalities in human iPSC-derived motor neurons

Homozygous ALS-linked mutations in TARDBP/TDP-43 lead to hypoactivity and synaptic abnormalities in human iPSC-derived motor neurons

Summary: Cytoplasmic mislocalization and aggregation of the RNA-binding protein TDP-43 is a pathological hallmark of the motor neuron (MN) disease amyotrophic lateral sclerosis (ALS). Furthermore, while mutations in TARDBP (encoding TDP-43) have been associated with ALS, the pathogenic consequences...

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Bibliographic Details
Main Authors: Sarah Lépine, Angela Nauleau-Javaudin, Eric Deneault, Carol X.-Q. Chen, Narges Abdian, Anna Krystina Franco-Flores, Ghazal Haghi, María José Castellanos-Montiel, Gilles Maussion, Mathilde Chaineau, Thomas Martin Durcan
Format: Article
Language:English
Published: Elsevier 2024-03-01
Series:iScience
Subjects:
Molecular neuroscience
Cellular neuroscience
Online Access:http://www.sciencedirect.com/science/article/pii/S2589004224003870

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http://www.sciencedirect.com/science/article/pii/S2589004224003870

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