{"product_id":"human-motor-neurons-ipsc-derived-sod1-mutant-a4v-hom-bhc18500136","title":"Human Motor Neurons (iPSC-derived, SOD1 mutant, A4V, HOM)","description":"\u003ch2\u003eOverview\u003c\/h2\u003e\n\u003cp\u003e\u003cstrong\u003eHuman Motor Neurons (iPSC-derived, SOD1 mutant, A4V, HOM)\u003c\/strong\u003e 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.\u003c\/p\u003e\n\u003cp\u003eSpinal 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.\u003c\/p\u003e\n\u003ch2\u003eKey elements and design rationale\u003c\/h2\u003e\n\u003cul\u003e\n  \u003cli\u003e\n\u003cstrong\u003eCell identity:\u003c\/strong\u003e Neural (iPSC-Derived Cells)\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eSource context:\u003c\/strong\u003e Motor Neurons; Nervous\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eDonor background:\u003c\/strong\u003e Disease\/condition: Amyotrophic Lateral Sclerosis\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eBiosafety level:\u003c\/strong\u003e BSL-2 (follow your institution’s biosafety program and local regulations)\u003c\/li\u003e\n\u003c\/ul\u003e\n\u003cp\u003eProduct-specific elements (such as tissue source, donor background, and cell classification) help frame how results should be interpreted across assays and experimental conditions.\u003c\/p\u003e\n\u003ch2\u003eBiological background\u003c\/h2\u003e\n\u003cp\u003eNeural and glial cell models support studies of neuronal signaling, synaptic biology, neuroinflammation, and cell-type–specific responses to injury or disease-relevant stimuli.\u003c\/p\u003e\u003cp\u003eAcross 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.\u003c\/p\u003e\n\u003ch2\u003eResearch relevance and current trends\u003c\/h2\u003e\n\u003cul\u003e\n  \u003cli\u003eIncreasing use of primary and specialty cells to improve translational relevance for target biology and phenotypic screening.\u003c\/li\u003e\n  \u003cli\u003eAdoption of 3D culture formats and co-culture systems to better capture tissue microenvironments and cell–cell interactions.\u003c\/li\u003e\n  \u003cli\u003eIntegration of functional readouts with single-cell and multi-omics profiling to connect phenotype with molecular state.\u003c\/li\u003e\n  \u003cli\u003eGrowth of human-relevant neural models (including glial components) to study circuit- and inflammation-linked phenotypes.\u003c\/li\u003e\n\u003c\/ul\u003e\n\u003ch2\u003eCommon research applications\u003c\/h2\u003e\n\u003cul\u003e\n  \u003cli\u003eProfile identity markers by flow cytometry or immunostaining in cultured cells\u003c\/li\u003e\n  \u003cli\u003eQuantify neurite outgrowth and synaptic marker profiles in neural cultures\u003c\/li\u003e\n  \u003cli\u003eQuantify functional responses to defined stimuli relevant to the model system\u003c\/li\u003e\n  \u003cli\u003eCompare baseline phenotype across donors\/conditions using gene expression profiling\u003c\/li\u003e\n  \u003cli\u003eMeasure neuroinflammatory signaling in neuron–glia or microglia-enriched models\u003c\/li\u003e\n\u003c\/ul\u003e\n\u003cp\u003eInterpretation 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.\u003c\/p\u003e\n\u003ch2\u003eNotes for experimental interpretation\u003c\/h2\u003e\n\u003cul\u003e\n  \u003cli\u003eDonor-to-donor heterogeneity can influence baseline phenotype and treatment response; include biological replicates when feasible.\u003c\/li\u003e\n  \u003cli\u003ePassage number, confluence, and media composition can shift gene expression and functional readouts; track and report these variables consistently.\u003c\/li\u003e\n  \u003cli\u003eContamination control (including routine mycoplasma monitoring) supports reproducibility in downstream assays.\u003c\/li\u003e\n  \u003cli\u003eUse appropriate negative\/positive controls for the readout (e.g., unstimulated controls, pathway agonists\/antagonists) to contextualize observed changes.\u003c\/li\u003e\n\u003c\/ul\u003e\n\u003c!-- Sources (internal):\n- ATCC Animal Cell Culture Guide — ATCC — https:\/\/www.atcc.org\/resources\/culture-guides\/animal-cell-culture-guide\n- Cell Line Authentication — ATCC — https:\/\/www.atcc.org\/resources\/culture-guides\/cell-line-authentication\n- Biosafety in Microbiological and Biomedical Laboratories (BMBL) — U.S. HHS\/CDC\/NIH — https:\/\/www.cdc.gov\/labs\/BMBL.html\n- Mycoplasma contamination in cell culture — NCBI Bookshelf\/PMC — https:\/\/www.ncbi.nlm.nih.gov\/pmc\/\n- Primary cell culture considerations — Nature Methods — https:\/\/www.nature.com\/nmeth\/\n- Good cell culture practice guidelines — OECD\/ECVAM (concept) — https:\/\/www.oecd.org\/\n--\u003e\n\u003cp style=\"display:none\"\u003eSKU:BHC18500136\u003c\/p\u003e","brand":"iXCells Biotechnologies","offers":[{"title":"Cryopreserved \/ 1 million cells\/vial","offer_id":53197810893165,"sku":"40HU-101-1M","price":1537.12,"currency_code":"USD","in_stock":true},{"title":"Cryopreserved \/ 2 million cells\/vial","offer_id":53197820035437,"sku":"40HU-101-2M","price":2344.16,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0949\/7424\/7277\/files\/40HU-101-1M.png?v=1775378645","url":"https:\/\/www.ebiohippo.com\/products\/human-motor-neurons-ipsc-derived-sod1-mutant-a4v-hom-bhc18500136","provider":"BioHippo","version":"1.0","type":"link"}