For research use only
| Cat No. | ABC-SC254Y |
| Product Type | Motor Neurons |
| Cell Type | Neural/Glial Cells |
| Species | Human |
| Growth Conditions | 37 ℃, 5% CO2 |
| Source Organ | Central Nervous System |
| Disease | ALS; Frontotemporal Dementia |
| Storage | Liquid Nitrogen |
Human iPSC-Derived Motor Neurons FUS P525L/P525L models ALS-associated motor neuron degeneration and FUS proteinopathy for neurodegenerative disease research.
HighQC Human iPSC-Derived Motor Neurons FUS P525L/P525L are human iPSC-derived motor neurons generated from a human iPSC line originally derived from adult skin fibroblasts. These cryopreserved cells provide a defined in vitro model for amyotrophic lateral sclerosis (ALS) and FUS-associated neurodegenerative disease research. The cells are produced through a directed differentiation protocol to support reproducible lineage-specific experimental systems. This line carries a homozygous FUS P525L missense mutation on both alleles, modeling FUS proteinopathy relevant to early-onset familial ALS, without implying a fixed clinical phenotype. They retain neuronal lineage characteristics associated with motor neuron models. Quality control testing includes sterility assessment and lineage characterization by protein expression and gene expression assays, where applicable. Each lot undergoes rigorous screening and isolation procedures, and is rigorously tested to ensure it is free of contamination from HIV-1, HBV, HCV, Syphilis, Mycoplasma, Fungi, Yeast, and Bacteria.
| Product Code | Motor Neurons FUS P525L/P525L; HighQC Human iPSC-Derived Motor Neurons FUS P525L/P525L; FUS |
| Species | Human |
| Cat.No | ABC-SC254Y |
| Product Category | Stem Cells |
| Size/Quantity | 1 vial |
| Cell Type | Neural/Glial Cells |
| Growth Mode | Adherent |
| Shipping Info | Dry Ice |
| Growth Conditions | 37 ℃, 5% CO2 |
| Source Organ | Central Nervous System |
| Disease | ALS; Frontotemporal Dementia |
| Biosafety Level | 1 |
| Storage | Liquid Nitrogen |
| Product Type | Motor Neurons |
| Quality Control | All cells test negative for mycoplasma, bacteria, yeast, and fungi. |
HighQC Human iPSC-Derived Motor Neurons FUS P525L/P525L are intended for research use in controlled in vitro workflows. They can be used for disease-relevant mechanism studies, genotype-associated pathway analysis, target validation, and candidate therapeutic evaluation in a human iPSC-derived motor neuron model. As a homozygous FUS proteinopathy model, this line supports studies of FUS cytoplasmic mislocalization and stress granule dynamics relevant to early-onset familial ALS, at the mechanism and assay-development level, not clinical or diagnostic use.