{"id":73686,"date":"2023-05-22T05:33:25","date_gmt":"2023-05-22T12:33:25","guid":{"rendered":"https:\/\/ixcellsbiotech.com\/product\/human-motor-neurons-ipsc-derived-amyotrophic-lateral-sclerosis-patient-sporadic\/"},"modified":"2026-07-14T14:26:29","modified_gmt":"2026-07-14T21:26:29","slug":"human-motor-neurons-ipsc-derived-amyotrophic-lateral-sclerosis-patient-sporadic","status":"publish","type":"product","link":"https:\/\/ixcellsbiotech.com\/product\/human-motor-neurons-ipsc-derived-amyotrophic-lateral-sclerosis-patient-sporadic\/","title":{"rendered":"Human Motor Neurons (iPSC-derived, Amyotrophic Lateral Sclerosis Patient, Sporadic)"},"content":{"rendered":"

Product Description<\/strong><\/span><\/span><\/strong><\/p>\n

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]<\/sup>. 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\u00a0<\/sup><\/span>[2,3]<\/sup>.<\/p>\n

iXCells Biotechnologies<\/strong> is proud to provide the world\u2019s 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)<\/a>. Moreover, whole cell patch clamp revealed that when cultured in Motor Neuron Activity Medium Kit (Cat# MD-0118-100ML)<\/a> 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.<\/p>\n

iXCells also provide customized differentiation service with your own iPS cell lines. \u00a0Please contact us at\u00a0orders@ixcellsbiotech.com<\/a>\u00a0for more details.<\/p>\n

\"motor<\/p>\n

\u00a0Figure 1 (A)<\/strong> Immunofluorescence staining showing HB9 and ChAT positive cells on day 2 and 7 in culture respectively. (B)<\/strong> Flow cytometry measurements demonstrate >85% HB9 and >90% ISL1 positive cells on day 1-2.<\/p>\n

\"\"<\/a><\/p>\n

\u00a0Figure 2 (A)<\/strong> An image of the cells during the experiment. The patch pipette is visible on the left. (B)<\/strong> The Sodium\/potassium currents were recorded in voltage clamp mode with test potentials of -100 mV to 90 mV. (C)<\/strong> Excitatory postsynaptic currents (EPSCs) were recorded in voltage clamp mode while clamping the cell at -60 mV. (D)<\/strong> 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)<\/strong> Spontaneous activity was recorded in current clamp mode with a current injection needed for a membrane potential of -45 mV.<\/p>\n

Product Details<\/strong><\/span><\/span><\/p>\n\n\n\n\n\n\n\n\n\n\n\n
Tissue Origin<\/strong><\/td>\nHomo Sapiens,<\/em> Human<\/td>\n<\/tr>\n
Cell Type<\/strong><\/td>\nNeuronal Cells<\/td>\n<\/tr>\n
Disease<\/strong><\/td>\nNormal<\/td>\n<\/tr>\n
Package Size<\/strong><\/td>\n1 x 106<\/sup> cells\/vial, 2 x 106<\/sup> cells\/vial and 4 x 106 <\/sup>cells\/vial<\/td>\n<\/tr>\n
Passage Number<\/strong><\/td>\nP0<\/td>\n<\/tr>\n
Growth Properties<\/strong><\/td>\nAdherent<\/td>\n<\/tr>\n
Product Format\/Shipped<\/strong><\/td>\nCryopreserved<\/td>\n<\/tr>\n
Storage<\/strong><\/td>\nLiquid Nitrogen<\/td>\n<\/tr>\n
Associated Media<\/strong><\/td>\niXCells\u2122 Motor Neuron Culture Medium Kit (Cat# MD-0022-100ML) <\/a><\/p>\n

iXCells\u2122 Motor Neuron Activity Medium Kit(Cat# MD-0118-100ML)<\/a><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n

References<\/span><\/span><\/strong><\/p>\n

[1] Brady ST. (1993). \u201cMotor neurons and neurofilaments in sickness and in health.\u00a0Cell<\/em>. 9;73(1):1-3.<\/p>\n

[2] Dolmetsch R, Geschwind DH. (2011) \u201cThe human brain in a dish: the promise of\u00a0iPSC-derived\u00a0neurons\u201d.\u00a0Cell<\/em>. 145(6):831-4.<\/p>\n

[3] Payne NL, Sylvain A, O’Brien C, Herszfeld D, Sun G, Bernard CC. (2015) \u201cApplication of human induced pluripotent stem cells for modeling and treating neurodegenerative diseases.\u201d New Biotechnology. 25;32(1):212-28.<\/p>\n

Datasheet & Culture Protocol<\/a><\/p>\n","protected":false},"excerpt":{"rendered":"

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, […]<\/p>\n","protected":false},"featured_media":90491,"template":"","meta":{"_acf_changed":false,"content-type":"","jetpack_publicize_message":"{title}\n\n{excerpt}\n\n{url}","jetpack_publicize_feature_enabled":true,"jetpack_social_post_already_shared":true,"jetpack_social_options":{"image_generator_settings":{"template":"highway","default_image_id":0,"font":"","enabled":false},"version":2},"_wpas_customize_per_network":false},"product_brand":[],"product_cat":[1835,1647],"product_tag":[2328,2273,2272,2327,2275,2332,2331,2227],"cell_type":[2420],"cell_system":[2426],"organ":[2439],"disease":[2459],"research_area":[],"mutation":[],"mutation_type":[],"disease_model":[],"parental_line_name":[],"class_list":["post-73686","product","type-product","status-publish","has-post-thumbnail","product_cat-diseases-modeling","product_cat-ipsc-ipsc-derived-cells","product_tag-als","product_tag-als-cells","product_tag-als-patient-derived","product_tag-amyotrophic-lateral-sclerosis-patient","product_tag-ipsc-als","product_tag-ipsc-derived","product_tag-remove-term-amyotrophic-lateral-sclerosis-patient-amyotrophic-lateral-sclerosis","product_tag-sporadic","cell_type-neurons-and-glial-cells","cell_system-nervous-system","organ-brain-central-nervous-system","disease-amyotrophic-lateral-sclerosis-als","first","instock","taxable","shipping-taxable","purchasable","product-type-variable"],"exclude_global_add_ons":[],"addons":[],"acf":[],"yoast_head":"\nHuman Motor Neurons (iPSC-derived, Amyotrophic Lateral Sclerosis Patient, Sporadic) - iXCells<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/ixcellsbiotech.com\/product\/human-motor-neurons-ipsc-derived-amyotrophic-lateral-sclerosis-patient-sporadic\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Human Motor Neurons (iPSC-derived, Amyotrophic Lateral Sclerosis Patient, Sporadic) - iXCells\" \/>\n<meta property=\"og:description\" content=\"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. 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