Article

Pathogenic TDP-43 Disrupts Axon Initial Segment Structure and Neuronal Excitability in a Human iPSC Model of ALS

2022-05-17

Abstract excerpt

Dysregulated neuronal excitability is a hallmark of amyotrophic lateral sclerosis (ALS). We sought to investigate how functional changes to the axon initial segment (AIS), the site of action potential generation, could impact neuronal excitability in a human iPSC model of ALS. We found that early (6-week) ALS-related TDP-43 G298S motor neurons showed an increase in the length of the AIS, relative to CRISPR-correc...

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Literature Corpus work: 0acc64af-4941-545a-9503-4c9e95e19fcc
DOI: 10.1101/2022.05.16.492186

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