cat no | io1046S
Within days, cells convert to a defined and scalable genetically matched system to study amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).
ioMotor Neurons TDP‑43 M337V/M337V are opti‑ox deterministically programmed motor neurons carrying a genetically engineered homozygous M337V mutation in the TARDBP gene, encoding TAR DNA binding protein 43 (TDP‑43).
Related disease model cells are available with a heterozygous TDP‑43 M337V/WT mutation, and both can be used alongside their genetically matched control, ioMotor Neurons.
Additional disease models are available in ioGlutamatergic Neurons with mutations in TDP‑43 and MAPT, creating a comprehensive toolkit to study the genetic and pathological overlap between ALS and FTD.
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.
Make True Comparisons
Pair the ioDisease Model Cells with genetically matched wild-type ioMotor Neurons to investigate the impact of mutant TDP-43 protein on disease progression.
Quick and easy
Within 4 days post revival cells are ready for experimentation, displaying motor neuronal morphology without clumping.
Defined
>80% cells express key lower motor neuron markers indicating a spinal motor neuron identity (cervical region). >99.9% neuronal population.
Schematic overview of the timeline in the user manual
ioMotor Neurons TDP-43 M337V/M337V form a homogenous neuronal network by day 4
ioMotor Neurons TDP‑43 M337V/M337V express motor neuron-specific markers with protein expression highly reminiscent to the genetically matched control
ioMotor Neurons TDP‑43 M337V/M337V demonstrate gene expression of neuronal-specific and motor neuron-specific markers following deterministic programming
Disease-related TARDBP is expressed in ioMotor Neurons TDP‑43 M337V/M337V following deterministic programming
Do more with every vial
Luke Foulser | Scientist | bit.bio
This poster presented at AD/PD 2023 shows FTD and ALS disease-related phenotypic data for ioGlutamatergic Neurons disease model cells carrying a mutation in MAPT or TDP-43 (TARDBP).