{"id":76719,"date":"2023-05-22T06:02:17","date_gmt":"2023-05-22T13:02:17","guid":{"rendered":"https:\/\/ixcellsbiotech.com\/product\/human-motor-neurons-ipsc-derived-tdp-43-mutation-q331k-het\/"},"modified":"2026-07-14T14:38:18","modified_gmt":"2026-07-14T21:38:18","slug":"human-motor-neurons-ipsc-derived-tdp43-mutation-q331k-het","status":"publish","type":"product","link":"https:\/\/ixcellsbiotech.com\/product\/human-motor-neurons-ipsc-derived-tdp43-mutation-q331k-het\/","title":{"rendered":"Human Motor Neurons (iPSC-derived, TDP-43 mutation, Q331K, HET)"},"content":{"rendered":"

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

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\u20135 years [1]<\/sup>. \u00a0iPSC-derived motor neurons are valuable tools for biochemical analysis, disease modelling and clinical application of this disease. \u00a0Cytoplasmic 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]<\/sup>. Over 40 dominantly inherited mutations in the gene encoding TDP-43 have subsequently been identified in familial ALS patients [4]<\/sup>, implicating TDP-43 dysfunction in the vast majority of ALS cases.<\/p>\n

Human Motor Neurons (iPSC-derived, TDP-43 mutation, Q331K) <\/strong>is derived from a genetically modified normal iPSC line carrying the heterozygous or homozygous Q331K mutation in the TDP-43 gene (Figure 1). iXCells\u2122 hiPSC-derived motor neurons express typical markers of motor neurons, e.g. HB9 (MNX1), ISL1, CHAT, (Figure 2), with the purity higher than 85%. iXCells\u2122 motor neurons are available in both cryopreserved vials and fresh plate formats (12, 24, 48, and 96-well plate). Most of the cells will express high level of HB9 and ISL-1 after thawing in the Motor Neuron Culture Medium Kit (Cat# MD-0022-100ML)<\/a>. After culturing in the medium for 5-7 days, these cells will express high levels of CHAT and MAP2.<\/p>\n

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

Figure 1<\/strong>.Heterozygous and homozygous Q331K mutation (highlighted) has been introduced to TDP-43 gene using CRISPR\/Cas9 based genome editing technology. The targeted site is verified by genomic PCR\/Sanger .sequencing.<\/p>\n

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

Figure 2<\/strong>.Immunofluorescence staining showing HB9 and ChAT positive cells on day 2 and 7 in culture respectively.<\/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>\nBrain Cell<\/td>\n<\/tr>\n
Disease<\/strong><\/td>\nALS<\/td>\n<\/tr>\n
Package Size<\/strong><\/td>\n1.0 million cells\/vial; 2.0 million cells\/vial<\/td>\n<\/tr>\n
Passage Number<\/strong><\/td>\nP0<\/td>\n<\/tr>\n
Growth Properties<\/strong><\/td>\nAdherent<\/td>\n<\/tr>\n
Shipped<\/strong><\/td>\nCryopreserved<\/td>\n<\/tr>\n
Storage<\/strong><\/td>\nLiquid Nitrogen<\/td>\n<\/tr>\n
Media<\/strong><\/td>\niXCells\u2122 Motor Neuron Culture Medium Kit (Cat# MD-0022-100ML)<\/a><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n

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

[1] Taylor, J. P., Brown, R. H. Jr & Cleveland, D. W. Decoding ALS: from genes to mechanism. Nature 539, 197\u2013206 (2016).<\/p>\n

[2] Neumann, M. et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314, 130\u2013133 (2006).<\/p>\n

[3] Ling, S. C., Polymenidou, M. & Cleveland, D. W. Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron 79, 416\u2013438 (2013).<\/p>\n

[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\u2013R64 (2010).<\/p>\n

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

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\u20135 years [1]. \u00a0iPSC-derived motor neurons are valuable tools for biochemical analysis, disease modelling and clinical application of this disease. \u00a0Cytoplasmic accumulation and nuclear 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