cat no | ioEA1006
ioGlutamatergic Neurons
TDP‑43 M337V/WT™
Human iPSC-derived ALS and FTD disease model
A rapidly maturing, consistent and scalable isogenic system to study amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).
ioGlutamatergic Neurons TDP‑43 M337V/WT are opti‑ox™ precision reprogrammed glutamatergic neurons carrying a genetically engineered heterozygous M337V mutation in the TARDBP gene, encoding TAR DNA binding protein 43 (TDP‑43).
Related disease model cells are available with a homozygous TDP‑43 M337V/M337V mutation, and both can be used alongside their genetically matched control, ioGlutamatergic Neurons™.
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Benchtop benefits
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.
Scalable
Industrial scale quantities are available with industry-leading seeding densities, and at a price point that allows the cells to be used from research to high throughput screening.
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.
Highly characterised and defined
ioGlutamatergic Neurons TDP‑43 M337V/WT express neuron-specific markers with protein expression highly reminiscent to the isogenic control
Immunofluorescent staining on post-revival day 11 demonstrates similar homogenous expression of pan-neuronal proteins MAP2 and TUBB3 (upper panel) and glutamatergic neuron-specific transporter VGLUT2 (lower panel) in ioGlutamatergic Neurons TDP‑43 M337V/WT compared to the isogenic control. 100X magnification.
ioGlutamatergic Neurons TDP‑43 M337V/WT form structural neuronal networks by day 11
ioGlutamatergic Neurons TDP‑43 M337V/WT mature rapidly and form structural neuronal networks over 11 days when compared to the isogenic control. Day 1 to 11 post-thawing; 100X magnification.
ioGlutamatergic Neurons TDP‑43 M337V/WT demonstrate gene expression of neuronal-specific and glutamatergic-specific markers following reprogramming
Gene expression analysis demonstrates that ioGlutamatergic Neurons TDP‑43 M337V/WT and the isogenic control (WT) lack the expression of pluripotency makers (NANOG and OCT4), at day 11, whilst robustly expressing pan-neuronal (TUBB3 and SYP) and glutamatergic-specific (VGLUT1 and VGLUT2) markers, and the glutamate receptor GRIA4. Gene expression levels were assessed by RT-qPCR (data expressed relative to the parental hiPSC control (iPSC Control), normalised to HMBS). Data represents day 11 post-revival samples.
Disease-related TARDBP is expressed in ioGlutamatergic Neurons TDP‑43 M337V/WT following reprogramming
Gene expression analysis demonstrates that ioGlutamatergic Neurons TDP‑43 M337V/WT and the isogenic control (WT) express the TARDBP gene encoding TDP‑43. Gene expression levels were assessed by RT-qPCR (data expressed relative to the parental hiPSC control (iPSC Control), normalised to HMBS). Data represents day 11 post-revival samples.
Cells arrive ready to plate
ioGlutamatergic Neurons TDP‑43 M337V/WT are delivered in a cryopreserved format and are programmed to rapidly mature upon revival in the recommended media. The protocol for the generation of these cells is a three-phase process: Induction, which is carried out at bit.bio (Phase 0), Stabilisation for 4 days (Phase 1), and Maintenance (Phase 2) during which the ioGlutamatergic Neurons TDP‑43 M337V/WT mature. Phases 1 and 2 after revival of cells are carried out at the customer site.
Industry leading seeding density
Do more with every vial
ioGlutamatergic Neurons TDP‑43 M337V/WT are compatible with plates ranging from 6 to 384 wells and are available in two vial sizes, tailored to suit your experimental needs with minimal waste.
The recommended seeding density is 30,000 cells/cm2, compared to up to 500,000 cells/cm2 for other available products on the market.
One small vial can plate a minimum of 0.7 x 24-well plate, 1 x 96-well plate, or 1.5 x 384-well plate. One Large vial can plate a minimum of 3.6 x 24-well plate, 5.4 x 96-well plate, or 7.75 x 384-well plates.
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Precision Cellular Reprogramming for Scalable and Consistent Human Neurodegenerative Disease Models
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Validation of ALS-relevant phenotypes in precision reprogrammed iPSC-derived glutamatergic Neurons containing a TDP-43 M337V mutation.
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