axoCells Human iPSC-Derived Motor Neurons (male, ALS, C9orf72 carrying)

Product Description

axoCells Human iPSC-Derived Motor Neurons for neuroscience research. Motor neurons made from iPSCs generated from a 62 year old asymptomatic ALS (C9ORF72 carrier) male donor's dermal fibroblasts. iPSC-Derived Motor Neurons of a sister, carrying the C9orf72 expansion but with ALS diagnosis, are also available.

• 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

The C9orf72 hexanucleotide repeat expansion of the parental iPSC line of the motor neuron progenitor was determined by Southern blot. C9orf72 is present in ~ 40% of familial ALS and 8-10 % of sporadic ALS. It is the most common mutation related to ALS – far more common than SOD1 or TDP-43. With our axoCells iPSC-derived motor neurons, you can unlock the benefits of iPSC technology for your in vitro neuromuscular disease models.

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.