ioMotor DM HERO RECT

cat no | io6019

ioMotor Neurons TDP-43 A382T/WT

Human iPSC-derived ALS and FTD disease model

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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.

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For academic discounts or bulk pricing inquiries, contact us

Human iPSC-derived ALS and FTD disease model

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 A382T/WT are opti‑ox deterministically programmed motor neurons carrying a genetically engineered heterozygous A382T mutation in the TARDBP gene, encoding TAR DNA binding protein 43 (TDP‑43). 

Related ioMotor Neurons disease model cells are available with a homozygous TDP‑43 M337V/M337V mutation, a heterozygous TDP-43 M337V/WT mutation or a heterozygous TDP-43 N352S/WT mutation. Which can all 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.

Benchtop benefits

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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.

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Quick and easy

Within 4 days post revival cells are ready for experimentation, displaying motor neuronal morphology without clumping.

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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 Neuron + DM timeline-1

ioMotor Neurons TDP-43 A382T/WT are delivered in a cryopreserved format and are programmed to rapidly mature upon revival in the recommended media.

Product specifications

Starting material

Human iPSC line

Seeding compatibility

6, 12, 24, 96 and 384 well plates

Shipping info

Dry ice

Donor

Caucasian adult male (skin fibroblast)

Vial size

Small: >1 x 10⁶ viable cells

Quality control

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

Differentiation method

opti-ox deterministic cell programming

Recommended seeding density

30,000 cells/cm²

User storage

LN2 or -150°C

Format

Cryopreserved cells

Product use

ioCells are for research use only

Applications

Neurodegeneration research
ALS & FTD disease modelling
Electrophysiological analysis
Drug development & discovery
Neuromuscular research
Neurotoxicology

Technical data

Ready within days

ioMotor Neurons TDP-43 A382T/WT form a homogenous neuronal network by day 4

ioMotorNeurons_TDP-43_A382T_Brightfield_morphology

ioMotor Neurons TDP-43 A382T/WT rapidly acquire a motor neuronal phenotype, forming homogenous neuronal networks, without clumping of cells. Compared to the genetically matched wild type control, ioMotor Neurons. Day 1 to 11 post thawing; 100X magnification.

Highly characterised and defined

ioMotor Neurons TDP‑43 A382T/WT express motor neuron-specific markers with protein expression highly reminiscent to the genetically matched control

ioMotorNeurons_TDP-43_A382T_mmunocytochemistry_Panel1
ioMotorNeurons_TDP-43_A382T_Immunocytochemistry_Panel2

Click on the tabs to explore the data.

Immunofluorescent staining on post-revival day 11 demonstrates similar homogenous expression of pan-neuronal proteins TUBB3 and MAP2, motor neuron specific markers ISL2 and HB9 and the cholinergic markers ChAT and VAChT in ioMotor Neurons TDP‑43 A382T/WT compared to the genetically matched control, ioMotor Neurons

ioMotor Neurons TDP‑43 A382T/WT demonstrate gene expression of neuronal-specific and motor neuron-specific markers following deterministic programming

ioMotorNeurons_TDP-43_A382T_RT-qPCR_Panel1

Gene expression analysis demonstrates that ioMotor Neurons TDP‑43 A382T/WT and the genetically matched control (WT) lack the expression of pluripotency makers (NANOG and OCT4), at day 11, whilst robustly expressing pan-neuronal (MAP2 and TUBB3), cholinergic (CHAT and VACHT) and motor neuron-specific (MNX1 and ISL2) markers. 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.

Industry leading seeding density

Do more with every vial

UPDATED ioMotor seeding graphic

The seeding density of our human iPSC-derived ioMotor Neurons and related disease models has been optimised and validated to a recommended seeding density of 30,000 cells/cm². This means scientists can do more with every vial and expand experimental design within budget without losing out on quality. Resulting in more experimental conditions, more repeats, and more confidence in the data. 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.

Technical data

Morphological assessments

ioMotor Neurons TDP-43 A382T/WT form a homogenous neuronal network by day 4

ioMotorNeurons_TDP-43_A382T_Brightfield_morphology

ioMotor Neurons TDP-43 A382T/WT rapidly acquire a motor neuronal phenotype, forming homogenous neuronal networks, without clumping of cells. Compared to the genetically matched wild type control, ioMotor Neurons. Day 1 to 11 post thawing; 100X magnification.

Immunocytochemistry analysis

ioMotor Neurons TDP‑43 A382T/WT express motor neuron-specific markers with protein expression highly reminiscent to the genetically matched control

ioMotorNeurons_TDP-43_A382T_mmunocytochemistry_Panel1
ioMotorNeurons_TDP-43_A382T_Immunocytochemistry_Panel2

Click on the tabs to explore the data.

Immunofluorescent staining on post-revival day 11 demonstrates similar homogenous expression of pan-neuronal proteins TUBB3 and MAP2, motor neuron specific markers ISL2 and HB9 and the cholinergic markers ChAT and VAChT in ioMotor Neurons TDP‑43 A382T/WT compared to the genetically matched control, ioMotor Neurons

Product resources

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Harnessing AI-guided visual biology to discover drug targets for neurodegenerative disease Webinar
Harnessing AI-guided visual biology to discover drug targets for neurodegenerative disease

Ben Bar-Sadeh, PhD | Senior Scientist | Anima Biotech

Tom Brown | Senior Product Manager | bit.bio

Watch now
ioMotor Neurons and related disease models | User Manual User manual
ioMotor Neurons and related disease models | User Manual
V6
2025
bit.bio
Download
MaxWell Summit 2024_Poster Presentation with Luke Foulser_ioMotor Neurons Video
MaxWell Summit 2024_Poster Presentation with Luke Foulser_ioMotor Neurons

Luke Foulser | Scientist | bit.bio

Watch
Rapid and consistent generation of functional motor neurons from reprogrammed human iPSCs using opti-ox technology Poster
Rapid and consistent generation of functional motor neurons from reprogrammed human iPSCs using opti-ox technology
Foulser, et al 
bit.bio
2024
Download

See phenotypic data on our ALS and FTD disease models

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).

ICC N279K MAP2

Expand your research

Click on the icons to find out more

ALS and FTD disease-relevant mutations such as SOD1, FUS, MAPT and TDP-43 to study with isogenic control in human iPSC-derived neurons.
Build neurodegeneration in vitro models
Modelling ALS and FTD with human iPSC-derived neurons
Expand your research
Build neurodegeneration in vitro models
Modelling ALS and FTD with human iPSC-derived neurons
ALS and FTD disease-relevant mutations such as SOD1, FUS, MAPT and TDP-43 to study with isogenic control in human iPSC-derived neurons.

Access ALS and FTD disease-relevant mutations such as SOD1, FUS, MAPT and TDP-43 (TARDBP) genetically engineered in ioGlutamatergic Neurons and ioMotor Neurons.

Find out more

CRISPRko-Ready Motor Neurons cells engineered to constitutively express Cas9 nuclease for the quick and easy generation of gene knockouts and CRISPR screens
Simplify gene knockouts
Use CRISPRko-Ready ioMotor Neurons cells to study your gene of interest
Expand your research
Simplify gene knockouts
Use CRISPRko-Ready ioMotor Neurons cells to study your gene of interest
CRISPRko-Ready Motor Neurons cells engineered to constitutively express Cas9 nuclease for the quick and easy generation of gene knockouts and CRISPR screens

Interested in gene knockouts and CRISPR screens?
CRISPRko-Ready ioMotor Neurons cells engineered to constitutively express Cas9 nuclease for the quick and easy generation of gene knockouts and CRISPR screens are currently under development.

Contact our team to be one of the first to try the product!

Mark-Kotter-headshot
Study ALS in complex cultures
Co-culture motor neurons with astrocytes to gain insights into electrical activity
Expand your research
Study ALS in complex cultures
Co-culture motor neurons with astrocytes to gain insights into electrical activity
Mark-Kotter-headshot
Study neuronal networks and the impact of ALS-disease-related mutations by co-culturing ioMotor Neurons with astrocytes. Access 14 disease models and the single co-culture protocol for MEA.

View the co-culture protocol
Explore
ALS & FTD Disease Models
ioAstrocytes
Engineer disease known mutations in human iPSC-derived neurons.
Custom cell development
Generate custom disease models or reporter lines
Expand your research
Custom cell development
Generate custom disease models or reporter lines
Engineer disease known mutations in human iPSC-derived neurons.

Build your custom disease model or reporter line to pair with wild-type ioMotor Neurons as the genetically matched control.
Throughout the custom process, our experts will bring your project to life, and be on hand to support you with any technical queries.

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Human iPSC-derived cells

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