iPSC motor neuron NMJ models for enhancing breakthroughs in ALS

The ALS NMJ Model Poster from Axol Bioscience showcases a next-generation in vitro platform for studying neuromuscular junctions using human iPSC-derived motor neurons and skeletal muscle cells. This model supports ALS research and drug discovery by enabling functional co-culture systems and electrophysiological analysis.

Key highlights include:

• Motor Neuron Characterization: iPSC-derived motor neurons from ALS patients (C9orf72, SOD1, TDP43) and healthy donors express TUJ1, HB9, ChAT, and ISL1; show spontaneous and synchronized burst firing via MEA.
• Skeletal Muscle Characterization: iPSC-derived myotubes express Actin, Titin, MyoD1, Myosin-HC, Myogenin, and Dystrophin; display sarcomere structure and multinucleation.
• Microfluidic NMJ Platform: DuaLink™ MEA-compatible system enables compartmentalized co-culture and imaging of motor neuron-driven muscle contraction.
• Hyperexcitability in ALS: C9orf72 motor neurons show increased activity and irregular bursts compared to unaffected controls; MEA reveals distinct electrophysiological signatures.
• Functional NMJ Formation: α-Bungarotoxin staining confirms synaptic connectivity; neurite extension and acetylcholine receptor clustering observed.
• Drug Screening Potential: RNASeq and TempoSeq analysis identify disease-relevant targets including KCNQ2/3, LARK2, NEDD4, ERBB2, and TBK1 across ALS, cancer, diabetes, and immunodeficiency.

Featured Axol Products:

• axoCells™ Motor Neurons – Healthy Donor – ax0076
• axoCells™ Motor Neurons – ALS (C9orf72) – ax0074
• axoCells™ Motor Neurons – ALS (SOD1) – ax0735
• axoCells™ Motor Neurons – ALS (TDP43) – ax0079