Human iPS Cell Line (Amyotrophic Lateral Sclerosis)

Human iPS Cell Lines (iPSCs) derived from Amyotrophic Lateral Sclerosis (ALS) patients are generated by reprogramming somatic cells, such as dermal fibroblasts or peripheral blood mononuclear cells, obtained from individuals diagnosed with ALS. These cell lines retain the specific genetic background and disease-associated mutations (e.g., in SOD1, C9orf72, TARDBP, or FUS) of the donor. Characterized by standard pluripotency markers (e.g., OCT4, NANOG, SOX2, SSEA-4) and a normal karyotype, they exhibit indefinite self-renewal and the capacity to differentiate into all three germ layers.

In culture, they grow as adherent, tightly packed colonies with a high nuclear-to-cytoplasmic ratio, typically maintained in a nutrient-supplemented basal medium under standard conditions (37°C, 5% CO₂). Their primary value lies in disease modeling; they can be directed to differentiate into motor neurons and glial cells, allowing researchers to study ALS pathogenesis, protein aggregation, and neural degeneration in a patient-specific human context. Consequently, they are widely used for high-throughput drug screening and the development of targeted therapies for this fatal neurodegenerative disorder.