Sartorius – iPSC-Derived Motor Neurons and Microglia From ALS Background Display Disease Phenotype

The whitepaper from Sartorius iPSC-Derived Motor Neurons and Microglia From ALS Background Display Disease Phenotype presents a robust in vitro model for ALS research using Axol Bioscience’s axoCells™ derived from C9orf72-mutant and healthy donor iPSCs. These models enable functional and morphological analysis of disease phenotypes relevant to neurodegeneration.

Key highlights include:

• axoCells™ Motor Neurons: Derived from ALS (C9orf72) and control iPSCs (ax0074 and ax0078), matured for 20 days. ALS neurons show disordered morphology, hyperexcitability, and reduced synchronicity in MEA and calcium imaging assays.
• axoCells™ Microglia: Differentiated from the same iPSC lines via mesoderm induction and macrophage precursors. ALS microglia exhibit reduced phagocytosis of myelin basic protein (MBP), especially post-cryopreservation.
• Electrophysiology & Imaging:
  • MEA recordings reveal irregular burst firing in ALS neurons.
  • Incucyte® Neuroburst Orange Lentivirus used for calcium flux imaging confirms increased burst rate and reduced mean correlation in ALS neurons.
• Immunocytochemistry Validation:
  • Motor neurons: TUJ1, HB9, LIM3, OLIG2
  • Microglia: TMEM119, P2RY12, CX3CR1, Iba1
• Functional Assays:
  • Phagocytosis measured using pHrodo®-labeled MBP and Incucyte® S3 system.
  • ALS microglia show statistically significant reduction in uptake compared to healthy controls.
• Co-Culture Potential: Enables study of motor neuron–microglia interactions from matched ALS backgrounds, supporting compound screening and personalized therapeutic development.

Featured Axol Products:

• axoCells™ Motor Neuron Progenitors – ax0074, ax0078
• axoCells™ Motor Neuron Accelerator – ax0072, ax0179
• axoCells™ Microglia
• axoCells™ Neural Media & Supplements