Modelling ALS and FTD

with human iPSC-derived neurons

 

Consistent, defined and scalable human iPSC-derived disease models for ALS and FTD research and drug discovery

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Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are closely related progressive and fatal neurodegenerative conditions for which there is no cure. One of the biggest challenges slowing down the pace of ALS and FTD drug discovery research is the lack of consistent, physiologically relevant human cell models that accurately represent disease phenotypes and human pathophysiology in vitro.

We have developed ioDisease Model Cells with the aim of solving these challenges.

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

Our TDP-43 and MAPT disease models show disease-related phenotypes of reduced neuronal activity and hyperphosphorylation respectively, when compared to a genetically matched control. ioDisease Model Cells are defined, consistent, and scalable, supporting translational research for the identification of new therapeutics that could halt or reverse the devastating effects of ALS and FTD.

Discover our ioDisease Model Cells for ALS and FTD research and drug discovery below.

ALS disease models in ioGlutamatergic Neurons and ioMotor Neurons

ioGlutamatergic Neurons TDP‑43 M337V/WT ioDisease Model Cells
ioGlutamatergic Neurons TDP‑43 M337V/WT cat no. ioEA1006
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ioGlutamatergic Neurons TDP‑43 M337V/M337V ioDisease Model Cells
ioGlutamatergic Neurons TDP‑43 M337V/M337V cat no. ioEA1005
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ioMotor Neurons TDP-43 M337V/WT ioDisease Model Cells
ioMotor Neurons TDP-43 M337V/WT cat no. io1050S
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ioMotor Neurons TDP-43 M337V/M337V ioDisease Model Cells
ioMotor Neurons TDP-43 M337V/M337V cat no. io1046S
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ioMotor Neurons SOD1 G93A/WT ioDisease Model Cells
ioMotor Neurons SOD1 G93A/WT cat no. io1042S
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ioMotor Neurons SOD1 G93A/G93A ioDisease Model Cells
ioMotor Neurons SOD1 G93A/G93A cat no. io1041S
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ioMotor Neurons FUS P525L/WT ioDisease Model Cells
ioMotor Neurons FUS P525L/WT cat no. io1055S
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ioMotor Neurons FUS P525L/P525L ioDisease Model Cells
ioMotor Neurons FUS P525L/P525L cat no. io1052S
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FTD disease models in ioGlutamatergic Neurons

ioGlutamatergic Neurons MAPT P301S/WT ioDisease Model Cells
ioGlutamatergic Neurons MAPT P301S/WT cat no. io1015
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ioGlutamatergic Neurons MAPT P301S/P301S ioDisease Model Cells
ioGlutamatergic Neurons MAPT P301S/P301S cat no. io1008
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ioGlutamatergic Neurons MAPT N279K/WT ioDisease Model Cells
ioGlutamatergic Neurons MAPT N279K/WT cat no. io1009
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ioGlutamatergic Neurons MAPT N279K/N279K ioDisease Model Cells
ioGlutamatergic Neurons MAPT N279K/N279K cat no. io1014
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Pair ioDisease Model Cells with a genetically matched wild-type control

ioGlutamatergic Neurons ioWild Type Cells
ioGlutamatergic Neurons cat no. io1001
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ioMotor Neurons ioWild Type Cells
ioMotor Neurons cat no. io1027
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See data on our functional, highly characterised ioMotor Neurons 

Watch our scientist and cellular phenotyping expert Luke Foulser present his poster on our ioMotor Neurons at the MaxWell Biosystems Summit 2024. Learn about the characterisation of these cells, including their electrophysiological functionality, and their indicated spinal cell identity and clump-free morphology.

Watch the poster presentation
Luke pointing at ioMotor Neurons poster at MaxWell Summit 2024

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).

Read the poster
ICC N279K MAP2

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 coding platform behind our cells