TDP-43 M337VWT ICC Overlay copy

cat no | ioEA1005 | homozygous mutation
cat no | ioEA1006 | heterozygous mutation

ioGlutamatergic Neurons
TDP-43 M337V

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-43M337V are opti-ox™ precision reprogrammed glutamatergic neurons carrying a genetically engineered M337V mutation in the TARDBP gene, encoding TAR DNA binding protein 43 (TDP-43), available with either a homozygous (M337V/M337V) or heterozygous (M337V/WT) genotype. Human stem cells, within days, convert consistently into mature, functional glutamatergic neurons that express pan-neuronal and glutamatergic markers TUBB3, MAP2 and VGLUT2, as well as disease-relevant TARDBP.


Wild-type ioGlutamatergic Neurons™ form the genetically matched control for the ioGlutamatergic Neurons TDP-43M337V disease model. This physiologically-relevant isogenic pairing offers a fast and easy-to-use next-generation model to investigate the impact of mutant TDP-43 protein on the disease progression.

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Benchtop benefits

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Easy-to-use

Cells are programmed to mature rapidly upon revival with a simple two-phase protocol and are ready for experimentation in just 11 days.

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Scalable

A choice of vial sizes enables use in small-scale experiments or high-throughput screening applications.

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Make True Comparisons

Pair the ioDisease Model Cells with the genetically matched wild-type ioGlutamatergic Neurons to investigate the impact of the mutation on disease progression.

Product information

Starting material

Human iPSC line

Karyotype

Normal (46, XY)

Seeding compatibility

6, 12, 24, 48, 96 & 384 well plates

Shipping info

Dry ice

Donor

Caucasian adult male (skin fibroblast)

Vial size

Small: >1 x 10 viable cells
Large: >5 x 10 viable cells

Quality control

Sterility, protein expression (ICC), genotype (Sanger seq) and gene expression (RT-qPCR)

Product use

These cells are for research use only

Differentiation method

opti-ox cellular reprogramming

Recommended seeding density

30,000 cells/cm2

User storage

LN2 or -150°C

Format

Cryopreserved cells

Applications

FTD and ALS research
Drug discovery and development
Disease modelling
High-throughput screening
Electrophysiological assays (MEA)
Co-culture studies

Genetic modification

M337V/M337V or M337V/WT mutation in the TARDBP gene

Technical data

Highly characterised and defined

ioGlutamatergic Neurons TDP-43 disease model cells express neuron-specific markers with protein expression highly reminiscent to the isogenic control

ioGlut-TDP43-ICC-1

Immunofluorescent staining on post-revival day 11 demonstrates similar homogenous expression of glutamatergic neuron-specific transporter VGLUT2 (upper panel) and pan-neuronal proteins MAP2 and TUBB3 (lower panel) in ioGlutamatergic Neurons TDP-43M337V/WT and ioGlutamatergic Neurons TDP-43M337V/M337V compared to the isogenic control. 100X magnification.

ioGlutamatergic Neurons TDP-43 disease model cells form structural neuronal networks by day 11

TDP-43_brightfield-website

ioGlutamatergic Neurons TDP-43M337V/WT and ioGlutamatergic Neurons TDP-43M337V/M337V 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 disease model cells demonstrate gene expression of neuronal-specific and glutamatergic-specific markers following reprogramming
ioGlut-TDP43-Expression_analysis-A-1
ioGlut-TDP43-Expression_analysis-B-1
Gene expression analysis demonstrates that ioGlutamatergic Neurons TDP-43M337V/WT, ioGlutamatergic Neurons TDP-43M337V/M337V 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 disease model cells following reprogramming
ioGlut-TDP43-TARDBP-A-1
ioGlut-TDP43-TARDBP-B-1
Gene expression analysis demonstrates that ioGlutamatergic Neurons TDP-43M337V/WT, ioGlutamatergic Neurons TDP-43M337V/M337V 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

ioGluts-TDP-43-M337V-horizontal-retina

ioGlutamatergic Neurons TDP-43M337V 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-43M337V 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
ioGlut-WT-well_plate-2

ioGlutamatergic Neurons TDP-43 disease model cells 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.

Product resources

Differentiating iPSC | which approach works best? Infographic
Differentiating iPSC | which approach works best?

bit.bio

Download
Modelling human neurodegenerative diseases in research & drug discovery Webinar
Modelling human neurodegenerative diseases in research & drug discovery

Dr Mariangela Iovino | Group Leader | Charles River
Dr Tony Oosterveen | Senior Scientist | bit.bio

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Partnering with Charles River to advance CNS drug discovery with ioGlutamatergic Neurons™

Charles River

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How to culture ioGlutamatergic Neurons™ Video tutorial
How to culture ioGlutamatergic Neurons™

Dr Kaiser Karim | Scientist
bit.bio

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How to prepare tissue culture vessels with PDL-Geltrex™ for ioGlutamatergic Neuron™ culture Video tutorial
How to prepare tissue culture vessels with PDL-Geltrex™ for ioGlutamatergic Neuron™ culture

Dr Kaiser Karim | Scientist
bit.bio

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Addressing current challenges of in vitro cell models 

Read this blog to find out how experts from across academia and industry are approaching the challenges of reproducibility of in vitro cell models as well as potential solutions.

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Wild Type and Isogenic Disease Model cells: A true comparison.

Further your disease research by pairing our wild type cells with isogenic disease models.

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