Investigation of early axonal phenotypes in an iPSC-derived ALS cellular model using a microfluidic device.

ABSTRACT:

Introduction

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease caused by the loss of upper and lower motor neurons. Mutations in the FUS/TLS gene have been reported as the second most common mutation in Japanese patients with familial ALS. In recent years, lower motor neurons (LMNs) differentiated from induced pluripotent stem cells (iPSCs) derived from ALS patients have been widely used to analyze the mechanisms of neuronal cell death and degeneration.

Methods

In this study, we developed a microfluidic device designed to observe axonal growth, morphology, and trafficking at high resolution in neurons derived from induced pluripotent stem cells (iPSCs) and tested whether our microfluidic device effectively evaluates neurodegenerative phenotypes.

SUBMITTER: Otomo A 

PROVIDER: S-EPMC12328293 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature