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

Overview

Human Motor Neurons (iPSC-derived, TDP43 mutation, N352S, HOM) is a cell model used for research applications where physiologically relevant identity and donor background support interpretation of experimental readouts. Human Neural associated with Amyotrophic Lateral Sclerosis within the Nervous system.

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) is derived from a genetically modified normal iPSC line carrying the heterozygous or homozygous N352S mutation in the TDP-43 gene (Figure 1). iXCells™ 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™ 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) . And after cultured in the medium for 5-7 days, these cells will express high levels of CHAT and MAP2. Figure 1 . Heterozygous and homozygous N352S 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. Figure 2 . Immunofluorescence staining showing HB9 and ChAT positive cells on day 2 and 7 in culture respectively.

Key elements and design rationale

• Cell identity: Neural (iPSC-Derived Cells)
• Source context: Motor Neurons; Nervous
• Donor background: Disease/condition: Amyotrophic Lateral Sclerosis
• Biosafety level: BSL-2 (follow your institution’s biosafety program and local regulations)

Product-specific elements (such as tissue source, donor background, and cell classification) help frame how results should be interpreted across assays and experimental conditions.

Biological background

Neural and glial cell models support studies of neuronal signaling, synaptic biology, neuroinflammation, and cell-type–specific responses to injury or disease-relevant stimuli.

Across primary and specialty cell models, experimental outcomes can be influenced by donor heterogeneity, passage history, confluence, and media composition. For interpretation, it is common to validate key markers or functional phenotypes in the user’s assay context and to document culture variables consistently.

Research relevance and current trends

• Increasing use of primary and specialty cells to improve translational relevance for target biology and phenotypic screening.
• Adoption of 3D culture formats and co-culture systems to better capture tissue microenvironments and cell–cell interactions.
• Integration of functional readouts with single-cell and multi-omics profiling to connect phenotype with molecular state.
• Growth of human-relevant neural models (including glial components) to study circuit- and inflammation-linked phenotypes.

Common research applications

• Profile identity markers by flow cytometry or immunostaining in cultured cells
• Quantify neurite outgrowth and synaptic marker profiles in neural cultures
• Quantify functional responses to defined stimuli relevant to the model system
• Compare baseline phenotype across donors/conditions using gene expression profiling
• Measure neuroinflammatory signaling in neuron–glia or microglia-enriched models

Interpretation typically focuses on how a perturbation (e.g., cytokine exposure, metabolic stress, genetic manipulation, or compound treatment) shifts marker profiles or functional readouts relative to an appropriate control matched for donor and culture variables.

Notes for experimental interpretation

• Donor-to-donor heterogeneity can influence baseline phenotype and treatment response; include biological replicates when feasible.
• Passage number, confluence, and media composition can shift gene expression and functional readouts; track and report these variables consistently.
• Contamination control (including routine mycoplasma monitoring) supports reproducibility in downstream assays.
• Use appropriate negative/positive controls for the readout (e.g., unstimulated controls, pathway agonists/antagonists) to contextualize observed changes.

SKU:BHC18500319

Customization & Add-ons: Can't find the cell line you need—or require a custom cell-based solution for your project? We can help you source the best match or support custom cell line services for diverse research needs, including cell line sourcing and selection (species, tissue, and disease model matching), stable cell line engineering (overexpression, knockdown, or knockout via CRISPR/Cas9, shRNA, or sgRNA), reporter gene integration (GFP, RFP, luciferase, and other fluorescent or bioluminescent constructs), genome editing and knockin (point mutations, tagged endogenous proteins, conditional alleles), inducible expression systems (Tet-On/Off and other regulatable constructs), drug resistance marker selection (puromycin, G418, hygromycin, and others), custom growth and media optimisation for specific assay requirements, scale-up production for high-throughput screening campaigns, and authentication and QC services (STR profiling, mycoplasma testing, viability assessment). Click Talk to a Scientist to submit a request, email us at support@biohippo.com, or explore our Research Services for additional support—our team will follow up with feasibility details and next steps.