Multielectrode array characterization of human iPSC-derived motor neuron disease model for ALS drug discovery

The application note Multielectrode Array Characterization of Human iPSC-Derived Motor Neuron Disease Model for ALS Drug Discovery presents Axol Bioscience’s rapid and reproducible workflow for generating functional ALS-relevant motor neurons.

Key highlights include:

• Donor Diversity:
  • 4 donor lines: 1 unaffected, 3 ALS (C9orf72, SOD1, TDP43)
• Workflow:
  • 20-day thaw-to-data protocol using Axol motor neurons and Accelerator supplement
  • Assay-ready neurons by Day 10 (unaffected) or Day 20 (ALS lines)
• Morphological Characterization:
  • Brightfield imaging shows distinct neurite outgrowth and network formation
  • ALS lines exhibit heterogenous morphologies and fibrous neurites
• Functional Assays:
  • MEA recordings via Axion Maestro Pro
  • ALS lines show hyperexcitability, altered burst patterns, and reduced synchrony
• Phenotypic Differences:
  • C9orf72: increased spike amplitude, shortened ISI, high network burst frequency
  • SOD1: decreased network burst %, fewer spikes per burst
  • TDP43: increased firing rate, long bursts, low synchrony
• Reproducibility:
  • Multiple production runs confirm batch-to-batch consistency
  • Functional QC integrated into standard workflows

This model supports high-throughput ALS drug screening with mutation-specific insights and robust functional validation.

Featured Axol Products:

• axoCells™ Motor Neurons – Unaffected Donor – ax0076
• axoCells™ Motor Neurons – ALS (C9orf72) – ax0074
• axoCells™ Motor Neurons – ALS (SOD1) – ax0735
• axoCells™ Motor Neurons – ALS (TDP43) – ax0079
• axoCells™ Motor Neuron Maintenance Medium – ax0072
• axoCells™ Motor Neuron Accelerator Supplement – ax0178
• axoCells™ ALS Bundle Kits – ax0184 (C9orf72), ax01835 (SOD1), ax0189 (TDP43), ax0186 (Unaffected)