Human Motor Neurons (iPSC-derived, TDP43 mutation, N352S, HET)

Description Product Code Price Quantity Add to Cart
Cryopreserved, 1.0 million cells/vial
40HU-102-1M
$1,133.00
Cryopreserved, 2.0 million cells/vial
40HU-102-2M
$1,727.00

 

Product Description

Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disease of the motor system, characterized by selective and progressive loss of motor neurons, eventually leading to paralysis and death within 2–5 years [1].  iPSC-derived motor neurons are valuable tools for biochemical analysis, disease modelling and clinical application of this disease.  Cytoplasmic accumulation and nuclear loss of the RNA binding protein transactive response DNA-binding protein 43 (TDP-43) from affected neurons in most instances of ALS [2-3]. Over 40 dominantly inherited mutations in the gene encoding TDP-43 have subsequently been identified in familial ALS patients [4], implicating TDP-43 dysfunction in the vast majority of ALS cases.

Human Motor Neurons (iPSC-derived, TDP-43 mutation, N352S, HET) is derived from a genetically modified normal iPSC line carrying the heterozygous N352S mutation in the TDP43 gene (Figure 1). iXCells™ hiPSC-derived motor neurons express typical markers of motor neurons, e.g. HB9 (MNX1), ISL1, CHAT, with the purity higher than 85%. 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 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.

human motor neurons n352n het

Figure 1. Heterozygous N352S mutation (highlighted in grey) and two silent mutations (yellow arrows) have been introduced to TDP-43 gene using CRISPR/Cas9 based genome editing technology. The targeted site is verified by genomic PCR/Sanger sequencing.

 

Product Details

  Tissue Origin

  Human iPSC-derived motor neurons (TDP-43 mutation, N352S, heterozygous)

  Package Size

  1.0 million cells/vial; 2.0 million cells/vial

  Shipped

  Cryopreserved 

  Media

  Human Motor Neuron Maintenance Medium (Cat# MD-0022)

 

References

[1] Taylor, J. P., Brown, R. H. Jr & Cleveland, D. W. Decoding ALS: from genes to mechanism. Nature 539, 197–206 (2016).

[2] Neumann, M. et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314, 130–133 (2006).

[3] Ling, S. C., Polymenidou, M. & Cleveland, D. W. Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron 79, 416–438 (2013).

[4] Lagier-Tourenne, C., Polymenidou, M. & Cleveland, D. W. TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum. Mol. Genet. 19, R46–R64 (2010).

 

 Download Datasheet

[1] Taylor, J. P., Brown, R. H. Jr & Cleveland, D. W. Decoding ALS: from genes to mechanism. Nature 539, 197–206 (2016).

[2] Neumann, M. et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314, 130–133 (2006).

[3] Ling, S. C., Polymenidou, M. & Cleveland, D. W. Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron 79, 416–438 (2013).

[4] Lagier-Tourenne, C., Polymenidou, M. & Cleveland, D. W. TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum. Mol. Genet. 19, R46–R64 (2010).

Biological
Species Homo sapiens