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These human spinal motor neurons (MNs) are derived from a genetically engineered induced Pluripotent Stem Cell (iPSC) line carrying a homozygous M337V mutation in the TARDBP gene, which encodes the TDP-43 protein. This mutation is strongly associated with familial and sporadic Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD). The cells are differentiated using a robust, floor-plate directed protocol to generate a highly pure population of functional motor neurons.

This isogenic model provides a precise and powerful tool for studying TDP-43 proteinopathy, a pathological hallmark of nearly all ALS cases. These neurons robustly recapitulate key disease features, including cytoplasmic TDP-43 mislocalization, stress granule dynamics, increased sensitivity to proteotoxic stress, and altered mitochondrial function. They are indispensable for dissecting the mechanisms of TDP-43-mediated neurodegeneration and for high-content screening of targeted therapeutics.

Cells are prepared and manufactured in an ISO 9001:2015 certified environment and ISO 5 cleanroom.