Human Motor Neurons (iPSC-derived, Diseased)

Description

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 orders@amogene.com 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).

 

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Product Details

Tissue	Human iPSC-derived motor neurons (Diseased – ALS)
Package Size	1.0 million cells/vial; 2.0 million cells/vial; 4.0 million cells/vial (frozen)
Shipped	Cryopreserved
Media	Human Motor Neuron Maintenance Medium (Cat # MD-0022)

 

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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.