cat no | ioEA1005
A rapidly maturing, consistent and scalable isogenic system to study amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).
ioGlutamatergic Neurons TDP‑43 M337V/M337V are opti‑ox deterministically programmed glutamatergic 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 isogenic control detected by microelectrode array (MEA) analysis.
Related disease model cells are available with a heterozygous TDP‑43 M337V/WT mutation, and both can be used alongside their genetically matched (isogenic) control, ioGlutamatergic Neurons.
Confidently investigate your phenotype of interest across multiple clones with our disease model clone panel. Detailed characterisation data (below) and bulk RNA sequencing data (upon request) help you select specific clones if required.
per vial
A maximum number of 20 vials applies. If you would like to order more than 20 vials, please contact us at orders@bit.bio.
Disease-related phenotype
MEA analysis detects lower weighted mean firing rate and network burst frequency compared to the wild‑type control.
Make True Comparisons
Pair the ioDisease Model Cells with the genetically matched wild-type ioGlutamatergic Neurons to investigate the impact of mutant TDP‑43 protein on disease progression.
Quick
The disease model cells and isogenic control are experiment ready as early as 2 days post revival, and form structural neuronal networks at 11 days.
ioGlutamatergic Neurons TDP‑43 M337V/M337V express neuron-specific markers with protein expression highly reminiscent to the isogenic control
ioGlutamatergic Neurons TDP‑43 M337V/M337V form structural neuronal networks by day 11
ioGlutamatergic Neurons TDP‑43 M337V/M337V demonstrate gene expression of neuronal-specific and glutamatergic-specific markers following deterministic programming
Disease-related TARDBP is expressed in ioGlutamatergic Neurons TDP‑43 M337V/M337V following deterministic programming
Do more with every vial
V1
2023
bit.bio | Axion BioSystems | Charles River Laboratories
Oosterveen, et al
bit.bio & Charles River Laboratories
2023
Madeleine Garrett | Field Application Specialist | bit.bio
Oosterveen, et al
bit.bio
2022
Dr Mariangela Iovino | Group Leader | Charles River
Dr Tony Oosterveen | Senior Scientist | bit.bio
Read this blog on glutamatergic neuron cell culture for our top tips on careful handling, cell plating and media changes to achieve success from the outset.
Further your disease research by pairing our wild type cells with isogenic disease models.