TDP-43 M337VWT ICC Overlay copy

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™.

Request a quote

Once your quote request has been submitted, one of our sales team will be in contact.

Unsure where to start?

Get in touch and we'll guide the way!

Benchtop benefits

defined_0

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_0

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_0

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.

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

Heterozygous M337V missense mutation in the TARDBP gene

Technical data

Highly characterised and defined

ioGlutamatergic Neurons TDP‑43 M337V/WT express neuron-specific markers with protein expression highly reminiscent to the isogenic control

ioGlutamatergic_neurons-TDP43-M337V-WT-ICC-VGLUT2

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-TDP43-M337V-WT-Morphology

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

ioGlutamatergic_neurons-TDP43-M337V-WT-rt-qPCR
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

ioGlutamatergic_neurons-TDP43-M337V-WT-rt-qPCR-TDP43
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-TDP43-M337V-WT-timeline-1

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
ioGlut-WT-well_plate-2

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.

Product resources

Precision Cellular Reprogramming for Scalable and Consistent Human Neurodegenerative Disease Models Talk
Precision Cellular Reprogramming for Scalable and Consistent Human Neurodegenerative Disease Models

Madeleine Garrett | Field Application Specialist | bit.bio

2023

Watch now
Genotype and phenotype validation of an isogenic human iPSC-derived neuronal model of Huntington’s Disease Poster
Genotype and phenotype validation of an isogenic human iPSC-derived neuronal model of Huntington’s Disease

V1
bit.bio

2022

Download
Validation of ALS-relevant phenotypes in precision reprogrammed iPSC-derived glutamatergic Neurons containing a TDP-43 M337V mutation. Poster
Validation of ALS-relevant phenotypes in precision reprogrammed iPSC-derived glutamatergic Neurons containing a TDP-43 M337V mutation.

V1
Charles River & bit.bio

2022

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

Charles River Laboratories &  bit.bio

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

Charles River

Watch now

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.

bit-bio_Addressing the challenges of cell models_Blog_Image1200x755

Related products

ioSkeletal Myocytes DMD Exon 52 Deletion™ ioDisease Model
ioSkeletal Myocytes DMD Exon 52 Deletion™ cat no. io1019
Order now
ioSkeletal Myocytes DMD Exon 44 Deletion™ ioDisease Model
ioSkeletal Myocytes DMD Exon 44 Deletion™ cat no. io1018
Order now
ioGlutamatergic Neurons PRKN R275W/R275W™ ioDisease Model
ioGlutamatergic Neurons PRKN R275W/R275W™ cat no. io1020
Order now
ioGlutamatergic Neurons PRKN R275W/WT™ ioDisease Model
ioGlutamatergic Neurons PRKN R275W/WT™ cat no. io1013
Order now
ioGlutamatergic Neurons GBA null/WT™ ioDisease Model
ioGlutamatergic Neurons GBA null/WT™ cat no. io1007
Order now
ioGlutamatergic Neurons TDP‑43 M337V/M337V™ ioDisease Model
ioGlutamatergic Neurons TDP‑43 M337V/M337V™ cat no. ioEA1005
Order now
ioSensory Neurons™ ioWild Type
ioSensory Neurons™ cat no. ioEA1024
Order now
ioGlutamatergic Neurons MAPT N279K/WT™ ioDisease Model
ioGlutamatergic Neurons MAPT N279K/WT™ cat no. io1009
Order now
ioGlutamatergic Neurons MAPT P301S/WT™ ioDisease Model
ioGlutamatergic Neurons MAPT P301S/WT™ cat no. io1015
Order now
ioGlutamatergic Neurons MAPT P301S/P301S™ ioDisease Model
ioGlutamatergic Neurons MAPT P301S/P301S™ cat no. io1018
Order now
ioMicroglia™ ioWild Type
ioMicroglia™ cat no. ioA021
Order now
ioGABAergic Neurons™ ioWild Type
ioGABAergic Neurons™ cat no. ioEA1003S
Order now
ioSkeletal Myocytes™ ioWild Type
ioSkeletal Myocytes™ cat no. io1002
Order now
ioGlutamatergic Neurons HTT 50CAG/WT™ ioDisease Model
ioGlutamatergic Neurons HTT 50CAG/WT™ cat no. ioEA1004
Order now
ioGlutamatergic Neurons™ ioWild Type
ioGlutamatergic Neurons™ cat no. io1001
Order now

Wild type and isogenic disease model cells: a true comparison.

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

bitbio-vials-Wild_and_Disease-staggered-2500px_wide

Related pages

Discover ioCells Learn about our range of human iPSC-derived cells for research and drug discovery
Resources Explore our latest scientific insights, webinars, blogs and videos
Our platform Discover the cell identity coding platform that powers our ioCells