Human Motor Neurons (iPSC-derived, SOD1 mutant, A4V, HOM)

Overview

Human Motor Neurons (iPSC-derived, SOD1 mutant, A4V, 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.

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. iPSC-derived motor neurons are valuable tools for biochemical analysis, disease modelling and clinical application of these diseases. Mutations (over 150 identified to date) in the SOD1 gene have been linked to familial ALS [1-3] . The most frequent mutations are A4V and H46R. A4V (alanine at codon 4 changed to valine) is the most common ALS-causing mutation in the U.S. population, with approximately 50% of SOD1-ALS patients carrying the A4V mutation [4-6] . Approximately 10 percent of all U.S. familial ALS cases are caused by heterozygous A4V mutations in SOD1. Human Motor Neurons (iPSC-derived, SOD1 mutant, A4V, HOM) is derived from a genetically modified normal iPSC line carrying the A4V mutation (Figure 1). iXCells™ hiPSC-derived motor neurons express typical markers of motor neurons, e.g. HB9 (MNX1), ISL1, CHAT, with the purity higher than 85%. 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 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 . A4V mutation (red arrow) and two silent mutations (green arrows) have been introduced to SOD1 gene using CRISPR/Cas9 based genome editing technology. The targeted site is verified by genomic PCR/Sanger sequencing.

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:BHC18500136

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