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High-throughput screening of ALS patient iPSC-derived spinal motor neurons identifies novel compounds that increase neurofilament light chain expression - PubMed

5 hours ago
  • #ALS
  • #iPSC
  • #drug discovery
  • ALS is a fatal neurodegenerative disease affecting motor neurons, with pathology including protein inclusions and neurofilament aggregates.
  • Current ALS treatments offer limited survival benefits, underscoring the need for new therapies.
  • Researchers developed a high-throughput screening system using ALS patient-derived iPSCs differentiated into spinal motor neurons.
  • Over 6,000 compounds were screened, identifying a novel compound that increases neurofilament light chain (NF-L) expression by more than 50%.
  • The initial compound inhibited TGF-β signaling, leading to optimization efforts.
  • A second compound was found that does not affect TGF-β signaling and shows better performance in drug metabolism and pharmacokinetics assays.
  • The study demonstrates the value of iPSC-derived neurons in disease modeling and drug discovery.