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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 Culture Medium Kit (Cat# MD-0022-100ML). Moreover, whole cell patch clamp revealed that when cultured in Motor Neuron Activity Medium Kit (Cat# MD-0118-100ML) over 65% of the neurons exhibited mature spiking, and over 35% of the neurons had spontaneous activity at a holding potential of -60 mV (Figure 2) indicating the presence of a highly mature population of neurons.

iXCells also provide customized differentiation service with your own iPS cell lines.  Please contact us at orders@ixcellsbiotech.com 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.

 Figure 2 (A) An image of the cells during the experiment. The patch pipette is visible on the left. (B) The Sodium/potassium currents were recorded in voltage clamp mode with test potentials of -100 mV to 90 mV. (C) Excitatory postsynaptic currents (EPSCs) were recorded in voltage clamp mode while clamping the cell at -60 mV. (D) Evoked action potentials were recorded in current clamp mode starting with a current injection 12 pA below what is needed to hold the neuron at – 60 mV and with 3pA current steps. (E) Spontaneous activity was recorded in current clamp mode with a current injection needed for a membrane potential of -45 mV.

Product Details

Tissue Origin Homo Sapiens, Human
Cell Type Neuronal Cells
Disease Normal
Package Size 1 x 106 cells/vial, 2 x 106 cells/vial and 4 x 10cells/vial
Passage Number P0
Growth Properties Adherent
Product Format/Shipped Cryopreserved
Storage Liquid Nitrogen
Associated Media iXCells™ Motor Neuron Culture Medium Kit (Cat# MD-0022-100ML)

 

iXCells™ Motor Neuron Activity Medium Kit(Cat# MD-0118-100ML)

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 & Culture Protocol

Technical Documentation

Citations

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 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).

Figure 3.  Co-culture of iXCells™ hiPSC-derived motor neurons with mouse astrocytes for 7 days. Motor neurons are stained with Tuj1, and mouse astrocytes are stained with GFAP.


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

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.

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