Your mutation of interest in our opti-ox powered human iPSC-derived cells
ioWild Type Cells
ioGlutamatergic Neurons
cat no | io1001
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ioWild Type Cells
ioSkeletal Myocytes
cat no | io1002
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ioWild Type Cells
ioMicroglia | Male
cat no | io1021
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ioWild Type Cells
ioGABAergic Neurons
cat no | io1003
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ioWild Type Cells
ioMotor Neurons
cat no | io1027
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Insights from our partners on Custom ioDisease Model Cells
"The access to better human cell-based in vitro disease models as generated within our partnership is transformational for the way we do drug discovery with our pharma and biotech clients. We can now provide discovery studies in human stem cell-based assays, where previously we would only do this in for example immortalised cell lines or more simple assay types.”
Marijn Vlaming, PhD
Charles River Laboratories
Huntington's disease
A Huntington's disease-relevant phenotype has been observed in ioDisease Model Cells developed for industry partners.
In this example, Charles River Laboratories carried out functional characterisation of ioGlutamatergic Neurons HTT 50CAG/WT by using MaxWell’s MaxTwo high-density microelectrode array (MEA) platform. The data demonstrates a significant decrease in firing rate compared to wild-type isogenic control.
Now, Charles River Laboratories use this disease model in their End-to-End Huntington’s Disease Studies offering. Their customers can access in vitro assays using a disease model that recapitulates the Huntington’s disease phenotype.
Now, Charles River Laboratories use this disease model in their End-to-End Huntington’s Disease Studies offering. Their customers can access in vitro assays using a disease model that recapitulates the Huntington’s disease phenotype.
Frontotemporal dementia (FTD)
Our disease models of tau dysfunction show a tau hyperphosphorylation phenotype important to frontotemporal dementia research.
A panel of disease models for studying frontotemporal dementia (FTD) was developed by engineering either FTD-causing heterozygous or homozygous N279K or P301S mutations in the MAPT gene of ioGlutamatergic Neurons.
At day 21 post-revival, the data showed hyperphosphorylation of mutant tau protein compared to the wild type control using high-content image analysis. The panel offers the potential to investigate the impact of mutant tau on the molecular mechanisms of disease and test potential drug candidates rapidly in high throughput multiwell plate format. Data courtesy of Charles River Laboratories.
Amyotrophic lateral sclerosis (ALS)
ioDisease Model Cells built to study ALS show a significantly reduced neuronal firing phenotype compared to the isogenic control.
Using a microelectrode array (MEA)-based assay our ALS disease model (ioGlutamatergic Neurons TDP-43 M337V/M337V=) demonstrates a significant reduction in neuronal activity compared to the ALS disease model with a heterozygous mutation (ioGlutamatergic Neurons TDP-43 M337V/WT) and the wild-type isogenic control. Data courtesy of Charles River Laboratories.
This indicates their potential as a relevant translational in vitro drug discovery model for ALS.
Charles River Laboratories Discovery team now offers these disease models alongside high-content imaging and gene expression analysis to support investigations into TDP-43 protein phosphorylation, mislocalisation, aggregation, and altered mRNA splicing, which are hallmarks of ALS and FTD.
Duchenne muscular dystrophy (DMD)
ASO-mediated Dystrophin restoration demonstrated in ioSkeletal Myocytes DMD Exon 44 Deletion disease model cells
The DMD disease model offers a valuable translational model to investigate methods for dystrophin restoration, such as ASO-mediated exon skipping.
In this example, ioSkeletal Myocytes (wild type control) and ioSkeletal Myocytes DMD Exon 44 Deletion (DMD Del Ex44) were treated by gymnosis with exon 45 skipping antisense oligonucleotide (ASO-1). The data shows a concentration-dependent increase in the amount of mRNA transcript (A) and dystrophin protein (B) in the DMD exon 44 deletion disease model cells, indicating that ASO-1 treatment has been successful in creating an in frame mRNA transcript for dystrophin that leads to protein expression. Data courtesy of Charles River Laboratories.
Interested in generating your own ioDisease Model Cells?
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Human iPSC-derived cells
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