axoCells Human iPSC-Derived Motor Neurons (female, ALS, TDP43)

Product Description

axoCells Human iPSC-Derived Motor Neurons (≥2 million cells) for neuroscience research. Motor neurons made from iPSCs generated from a 62 year old symptomatic ALS (TDP43) female donor's dermal fibroblasts.

• Assay-ready in just 10 days
• Expression of the key markers including HB9, MAP2, LIM3 and ChAT2
• Designed for advanced in vitro model formats including co-culture and organ-on-chip platforms
• Manufactured in our ISO 9001-accredited production facility with excellent ISSCR Standards compliance

axoCellsTM motor neurons have been specifically developed for use in in vitro disease modeling and drug discovery. As a neuromuscular junction (NMJ) model with iPSC derived skeletal muscle, the ALS, unaffected ALS and healthy motor neurons have been used in both 2D and 3D formats to characterize and measure structural and functional phenotypic responses in vitro.   

A community of top ten biopharma and research institutes uses Axol's motor neurons to build powerful in vitro models, investigating neurodegenerative disorders such as ALS and for drug screening.

Supporting Data

ALS-derived motor neurons form smaller, less uniform bodies with thinner neurite extensions compared to healthy axoCells motor neurons.

Figure 1. Phase contrast images of mature axoCells motor neurons (day 21) from healthy (left, ax0078) and ALS (right, ax0074) background.

Compared to healthy axoCells motor neurons, ALS neurons form smaller, less uniform bundles, with thinner neurites. Healthy axoCells motor neurons form large, thick cabling between uniformly clustered bundles.

Figure 2. Immunocytochemistry of healthy control-derived axoCells motor neurons (top) and ALS-derived axoCells motor neurons (bottom) at day 21. Composite image for each channel and merged. Image captured on a Leica microscope at x20 magnification.

axoCells motor neurons exhibit synchronized burst firing, demonstrating their functional relevance.

Figure 3. axoCells motor neuron firing assessed at day 10 using the Axion Maestro Pro multi-electrode array (MEA) system. Here you can see sodium spike firing and network burst firing responses of the motor neurons.
Left: sodium spike profile, right: Raster plot showing burst firing events (blue boxes) with synchronized firing highlighted in pink boxes.