Description
Build high-quality in vitro neuromuscular disease models in 10 days.
Ensure confidence in your workflow with high-quality axoCellsTM motor neurons differentiated from human induced pluripotent stem cells (iPSCs). Derived from consenting donor fibroblasts, our motor neurons have been specifically developed for use in powerful in vitro monoculture and co-culture models, microfluidics platforms and for drug discovery.
With Axol, you can rely on robust, physiologically relevant motor neurons that express the key markers (HB9, MAP2, LIM3 and ChAT2) and are assay-ready in just 10 days using our Motor Neuron Maturation Accelerator Supplement. That means robust, physiologically relevant models of diseases such as ALS.
Supporting quality and consistency
Confidently carry out your neural workflow with iPSCs manufactured in our ISO:9001-accredited production facility, guided by our rigorous quality control procedures and decades of scientific experience. All cells come with a full Certificate of Analysis and are officially certified by HPSCreg®, to ensure ethical and biological conformity for your peace of mind.
Join our customer base of top ten biopharma and academic institutions in building robust, physiologically relevant disease models for research and drug discovery.
How to get highly functional axoCells Motor Neurons
To ensure simplicity and effectiveness, we’ve developed a ‘kit’ containing iPSC-derived cells that have been directed down a motor neuron end-type lineage. Using the easy-to-follow protocol, you can rapidly mature these cells into functional motor neurons in just 10 days.
- You’ll need a Motor Neuron Kit ax0186
- In this you’ll find Motor Neuron Cells ax0076 and all reagents*
- Follow the protocol
- 10 days later you will have highly functional motor neurons
*Additional third party components are required. Be sure to check the protocol before you start using the motor neuron kit.
axoCells motor neurons
| Donor | Age at sampling | Details | Mutation | Material & Reprogramming method | Cells | Bundle Kit |
| Unaffected donor | 40-50 | Male | None | Fibroblast / Sendai | ax0076 | ax0186 |
| C9orf72 carrying donor | 62 | Male, sibling of ax0074. Caucasian / Ashkenazi Familial history of ALS and dementia | C9orf72 repeat expansion | Fibroblast / Sendai | ax0073 | ax0183 |
| ALS donor | 64 Onset of ALS at 60 – death at 65. | Female, sibling of ax0073. Caucasian / Ashkenazi Familial history of ALS and dementia | C9orf72 repeat expansion | Fibroblast / Sendai | ax0074 | ax0184 |
| 61 | Female | SOD1 Het D109Y (G>T) | Fibroblast / Sendai | ax0735 | ax01835 | |
| 62 | Female | TDP43 A382T | Fibroblast / Sendai | ax0079 | ax0189 | |
Cells supplied ≥2 million precursor motor neuron cells, supplied frozen.
Bundle kit contains cells, media and Accelerator, BDNF, GDNF, CNTF. Other third-party reagents required.
For a quotation email operations@axolbio.com
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Phenotypic characterization: morphology
axoCells motor neurons demonstrate expected physiological morphology with uniform cell clusters and large cabling neuronal networks.
Phase contrast images of axoCells motor neurons matured from progenitors over 21 days.
Phenotypic characterization: ICC
axoCells motor neurons express key markers (TUJ1, ChAT) demonstrating phenotypic relevance.
Immunocytochemistry of day 21 mature axoCells motor neurons demonstrating presence of key markers (ChAT and TUJ1). Images captured on a Leica microscope x20 magnification. Error bar= 200um
Functional relevance: MEA
axoCells motor neurons exhibit synchronized burst firing, demonstrating their functional relevance
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.
Functional relevance: SNA (spontaneous neural activity)
axoCells motor neurons exhibit regular, synchronized firing on calcium imaging, demonstrating their functional relevance.
Firing pattern of axoCells motor neurons at day 21, transfected with NeuroBurst (calcium-sensitive lentivirus driven off the synapsin reporter) to pick up spontaneous neuronal activity on an IncuCyte. This demonstrates regular, synchronized firing with a mean correlation of 0.92. Potential parameters measured include mean correlation, burst rate, burst duration and burst strength.
Disease line vs control: ALS phase contrast
ALS-derived motor neurons form smaller, less uniform bodies with thinner neurite extensions compared to healthy axoCells motor neurons.
Phase contrast images of mature axoCells motor neurons (day 21) from healthy (left, ax0078) and ALS (right, ax0074) background.
Disease line vs control: ALS ICC
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.
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
Disease line vs control: SNA
Compared to healthy axoCells motor neurons, ALS-derived motor neurons exhibit less frequent and less-synchronized firing.
axoCells healthy control and ALS-derived motor neurons were matured for 21 days and transfected with Neuroburst Orange to look at spontaneous neuronal activity on the IncuCyte S3. Healthy motor neurons show regular synchronous firing compared to ALS which fire more frequently, for shorter duration and in a less-synchronized manner (shown by burst duration and rate). N=3, ***p<0.001, ****p<0.0001
axoModels: neuromuscular junction (NMJ)
Here is an example of a complex model we have built with motor neurons. As part of our axoModels offering, we are collaborating with biopharma organizations to build complex in vitro models, including an NMJ.
axoCells motor neurons matured on a microfluidics device with skeletal muscle in the opposite chamber. Neurite extensions can be observed passing through the grooves and innervating the skeletal muscle to form functional neuromuscular junctions (shown by bungarotoxin staining). A 3D model (not shown) has also been developed using a scaffold for the motor neurons, which are placed on top of skeletal muscle.
Applications
Our 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, our 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.
We have a community of top ten biopharma and research institutes that use our motor neurons to build powerful in vitro models, investigating neurodegenerative disorders such as ALS and for drug screening
Protocols
A fully validated protocol for accelerated maturation of motor neurons is available here.
Quality
We maintain or exceed industry-level quality with our ISO:9001-accredited production facility, guided by our rigorous quality control procedures and decades of scientific experience.
All cells come with a full Certificate of Analysis and are officially certified by HPSCreg®, to ensure ethical and biological conformity for your peace of mind.
Licenses & Consent
Axol has obtained all relevant licenses for reprogramming donor samples into iPSCs and differentiating these into motor neurons for commercial use.
Patient samples used to create these neurons have been ethically sourced and consented for research and commercial use. All cells come with a full Certificate of Analysis and are officially certified by HPSCreg®, to ensure ethical and biological conformity for your peace of mind.
McCallister TX et al. A high-fidelity CRISPR-Cas13 system improves abnormalities associated with C9ORF72-linked ALS/FTD. bioRxiv (2023) [Preprint]. doi: 10.1101/2023.12.12.571328.
Spijkers, X.M., Pasteuning-Vuhman, S., Dorleijn, J.C. et al. A directional 3D neurite outgrowth model for studying motor axon biology and disease. Sci Rep 11, 2080 (2021). https://doi.org/10.1038/s41598-021-81335-z
Fiskum Vegard, Sandvig Axel, Sandvig Ioanna 2021. Silencing of Activity During Hypoxia Improves Functional Outcomes in Motor Neuron Networks in vitro. Frontiers in Integrative Neuroscience 15 2021. DOI=10.3389/fnint.2021.792863
Rimington, R.P., Fleming, J.W., Capel, A.J. et al. Bioengineered model of the human motor unit with physiologically functional neuromuscular junctions. Sci Rep 11, 11695 (2021). https://doi.org/10.1038/s41598-021-91203-5
Media and Reagents
An optimized media and all growth factors are available to mature motor neurons in culture within 10 days of thaw.
