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

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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.

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.

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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.

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