Human iPSC-derived ALS and FTD disease model
A rapidly maturing, consistent and scalable system to study amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).
ioGlutamatergic Neurons TDP‑43 M337V/M337V are opti‑ox deterministically programmed excitatory neurons carrying a genetically engineered homozygous M337V mutation in the TARDBP gene, encoding TAR DNA binding protein 43 (TDP‑43).
The disease model cells show a phenotype of reduced neuronal activity compared to the genetically matched control detected by microelectrode array (MEA) analysis.
Related disease model cells are available with a heterozygous TDP‑43 M337V mutation, and both can be used alongside their genetically matched (isogenic) control, ioGlutamatergic Neurons.
Applications: FTD and ALS research, Drug discovery and development, Disease modelling, High-throughput screening, Electrophysiological assays (MEA), Co-culture studies