Human Motor Neurons (iPSC-derived, Normal)

Description	Product Code	Price
Cryopreserved, 1.0 million cells/vial	40HU-005-1M	$852.00
Cryopreserved, 2.0 million cells/vial	40HU-005-2M	$1,633.00
Cryopreserved, 4.0 million cells/vial	40HU-005-4M	$2,203.00

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 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 Co-culture Medium (Cat# MD-0023). 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 This email address is being protected from spambots. You need JavaScript enabled to view it. for more details.

motor neurons 1

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.

Product Details

Tissue	Human iPSC-derived motor neurons (Normal)
Package Size	1.0 million cells/vial; 2.0 million cells/vial; 4.0 million cells/vial (frozen)
Shipped	Cryopreserved
Storage	Liquid Nitrogen
Media	Motor Neuron Co-culture Medium (Cat# MD-0023) Motor Neuron Recovery Supplement (Cat# MD-0115)

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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.

Datasheet

Protocol

Wong, J. K., Roselle, A. K., Shue, T. M., Shimshak, S. J., Beaty, J. M., Celestin, N. M., Gao, I., Griffin, R. P., Cudkowicz, M. E., & Sadiq, S. A. (2022). Apolipoprotein B-100-mediated motor neuron degeneration in sporadic amyotrophic lateral sclerosis. Brain Communications, 4(4). https://doi.org/10.1093/braincomms/fcac207 -- Learn More

Liu B, Li M, Zhang L, Chen Z, Lu P. (2022). Motor neuron replacement therapy for amyotrophic lateral sclerosis. Neural Regen Res;17:1633-9 -- Learn More

Liu, Y., Dodart, J., Tran, H., Berkovitch, S., Braun, M., Byrne, M., . . . Brown, R. H. (2021). Variant-selective stereopure oligonucleotides protect against pathologies associated with c9orf72-repeat expansion in preclinical models. Nature Communications, 12(1). doi:10.1038/s41467-021-21112-8 -- Learn More
Shen, X., Beasley, S., Putman, J. N., Li, Y., Prakash, T. P., Rigo, F., . . . Corey, D. R. (2019). Efficient electroporation of neuronal cells using synthetic oligonucleotides: Identifying duplex RNA and antisense oligonucleotide activators of Human frataxin expression. RNA, 25(9), 1118-1129. doi:10.1261/rna.071290.119 -- Learn More
Gasset-Rosa, F., Lu, S., Yu, H., Chen, C., Melamed, Z., Guo, L., . . . Cleveland, D. W. (2019). Cytoplasmic TDP-43 de-mixing independent of stress granules drives inhibition of nuclear import, loss of nuclear TDP-43, and cell death. Neuron, 102(2). doi:10.1016/j.neuron.2019.02.038 -- Learn More
Martier, R., Liefhebber, J. M., García-Osta, A., Miniarikova, J., Cuadrado-Tejedor, M., Espelosin, M., . . . Konstantinova, P. (2019). Targeting rna-mediated toxicity in c9orf72 als and/or ftd by rnai-based gene therapy. Molecular Therapy - Nucleic Acids, 16, 26-37. doi:10.1016/j.omtn.2019.02.001 -- Learn More
Melamed, Z., López-Erauskin, J., Baughn, M. W., Zhang, O., Drenner, K., Lin, N., Wu, D., . . . Cleveland, D. W. (2019). Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of tdp-43-dependent neurodegeneration. Nature Neuroscience, 22(2), 180-190. doi:10.1038/s41593-018-0293-z -- Learn More
Marei, H. E., Althani, A., Lashen, S., Cenciarelli, C., & Hasan, A. (2017). Genetically unmatched human Ipsc and Esc Exhibit Equivalent gene expression and neuronal differentiation potential. Scientific Reports, 7(1). doi:10.1038/s41598-017-17882-1 -- Learn More

Danziger, S. A., Miller, L. R., Singh, K., Whitney, G. A., Peskind, E. R., Li, G., . . . Smith, J. J. (2017). An indicator cell assay for blood-based diagnostics. PLOS ONE, 12(6). doi:10.1371/journal.pone.0178608 -- Learn More