Human Motor Neurons (iPSC-derived, Amyotrophic Lateral Sclerosis Patient, Sporadic)
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 . 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.
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.
|Human iPSC-derived motor neurons (Diseased - ALS)|
|1.0 million cells/vial; 2.0 million cells/vial; 4.0 million cells/vial (frozen)|
|Human Motor Neuron Maintenance Medium (Cat # MD-0022)|
 Brady ST. (1993). “Motor neurons and neurofilaments in sickness and in health. Cell. 9;73(1):1-3.
 Dolmetsch R, Geschwind DH. (2011) “The human brain in a dish: the promise of iPSC-derived neurons”. Cell. 145(6):831-4.
 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.
Osaki, T., Uzel, S. G., & Kamm, R. D. (2018). Microphysiological 3D model of amyotrophic lateral sclerosis (ALS) from human iPS-derived muscle cells and optogenetic motor neurons. Science Advances, 4(10). doi:10.1126/sciadv.aat5847 -- Learn More
|Species||Homo sapiens (Diseased)|