Human Motor Neurons (iPSC-derived, SOD1 mutant, A4V)

Product Description

Spinal motor neurons (MNs) are a highly specialized type of neurons that reside in the ventral horns and project axons to muscles to control their movement. Degeneration of MNs is implicated in a number of devastating diseases, including spinal muscular atrophy (SMA), amyotrophic lateral sclerosis (ALS), Charcot-Marie-Tooth and poliomyelitis disease ^[1].  iPSC-derived motor neurons are valuable tools for biochemical analysis, disease modelling and clinical application of these diseases ^[2,3].

iXCells Biotechnologies is proud to provide iXCells™ motor neurons derived from normal donors or ALS patients. iXCells™ motor neurons express typical markers of motor neurons, e.g. HB9 (MNX1), ISL1, CHAT (Figure 1 and Figure 2), with the purity higher than 85%. Functional validation of iPSC-derived motor neurons has been done with neuromuscular junction formation (Video 1-4).

iXCells™ motor neurons are available in both cryopreserved vials (2 million cells/vial) and fresh plate formats (12-well plate or 96-well plate). Most of the cells will express high level of HB9 and ISL-1 (Figure 1) after thawing in the Motor Neuron Maintenance Medium (Cat# MD-0022). And after cultured in the medium for 5-7 days, these cells will express high levels of CHAT and MAP2 (Figure 2). 

iXCells also provide customized differentiation service with your own iPS cell lines.  Please contact us at This email address is being protected from spambots. You need JavaScript enabled to view it. for more details.

Figure 1.  After cultured in Motor Neuron Maintenance Medium on the Matrigel-coated plates for 2 days, more than 85% of the iPSC-derived motor neurons express HB9 (Figure A and A’),  and more than 90% of the cells express ISL1 (Figure B and B’). 

Figure 2.  After cultured in Motor Neuron Maintenance Medium on the Matrigel-coated plates for 5-7 days, more than 85% of the iPSC-derived motor neurons express ChAT (Figure A) and MAP2 (Figure B). 

Product Details

References

[1] Brady ST. (1993). “Motor neurons and neurofilaments in sickness and in health. Cell. 9;73(1):1-3. 

[2] Dolmetsch R, Geschwind DH. (2011) “The human brain in a dish: the promise of iPSC-derived neurons”. Cell. 145(6):831-4.

[3] Payne NL, Sylvain A, O'Brien C, Herszfeld D, Sun G, Bernard CC. (2015) “Application of human induced pluripotent stem cells for modeling and treating neurodegenerative diseases.” New Biotechnology. 25;32(1):212-28.

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