Human Motor Neurons (iPSC-derived, Amyotrophic Lateral Sclerosis Patient, Sporadic)

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. Neurodegenerative diseases, such as spinal muscular atrophy (SMA), amyotrophic lateral sclerosis (ALS), Charcot-Marie-Tooth and poliomyelitis disease are a result of the progressive degeneration of motor neurons ⁽¹⁾. Furthermore, motor neurons derived from normal, or patient induced pluripotent stem cells (iPSCs) enable the generation of cell models with features relevant to human physiology, thus making it a valuable tool for biochemical analysis, disease modelling and other broad range of clinical applications ^(2,3) .

iXCells Biotechnologies is proud to provide the world’s first fully differentiated and functional human iPSC-derived motor neurons that display typical neuronal morphology and express all key markers of motor neurons, e.g., HB9 (MNX1), ISL1, ChAT (Figure 1) when cultured in the Motor Neuron Maintenance Medium (Cat# MD-0022). In addition, our iPSCderived motor neurons can also be co-cultured with myotubes or glial cells for drug screening platforms.

iXCells also provide customized differentiation service with your own iPS cell lines.  Please contact us at [email protected] for more details.

Figure 1 (A) Immunofluorescence staining showing HB9 and ChAT positive cells on day 2 and 7 in culture respectively. (B) Flow cytometry measurements demonstrate >85% HB9 and >90% ISL1 positive cells on day 1-2.

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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.