Key Points:

• Development of a Novel Protocol for Efficient Induction of Lower Motor Neurons
  We have developed a highly efficient protocol for the induction of lower motor neurons from human iPSCs, achieving an induction efficiency of approximately 80%. Compared to conventional methods, this approach accelerates differentiation while maintaining high reproducibility, making it a valuable tool for studying ALS pathology and drug screening.

• Replication ALS pathology
  Lower motor neurons derived from iPSCs of familial ALS patients successfully recapitulated ALS-specific pathological features, including abnormal aggregation of TDP-43 and FUS proteins. These results highlight the usefulness of our method as a reliable model for ALS research.

• Validation of Functional Neuronal Activity
  The induced lower motor neurons exhibited mature neuronal activity as evidenced by firing patterns and network behavior, as confirmed by high density multielectrode array (HD-MEA) system analysis.

• Assessment of ALS Cell Vulnerability
  Using live time-lapse imaging, single cell tracking and AI-based image analysis, we showed that lower motor neurons from ALS patients, including a sporadic ALS patient, had reduced survival rates compared to those from healthy controls. This trend was not detectable using traditional imaging methods that rely on simple automated cell body counting, highlighting the advanced capabilities of our technology for assessing cell vulnerability in ALS research.

Pressrelease
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