cat no | io1055
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.
Make True Comparisons
Pair the ioDisease Model Cells with genetically matched wild-type ioMotor Neurons to investigate the impact of mutant FUS protein on disease progression.
Quick and easy
Within 4 days post revival cells are ready for experimentation, displaying motor neuronal morphology without clumping.
Defined
>80% cells express key lower motor neuron markers indicating a spinal motor neuron identity (cervical region). >99.9% neuronal population.
ioMotor Neurons FUS P525L/WT are delivered in a cryopreserved format and are programmed to rapidly mature upon revival in the recommended media.
ioMotor Neurons FUS P525L/WT form a homogenous neuronal network by day 4
ioMotor Neurons FUS P525L/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.
ioMotor Neurons FUS P525L/WT express motor neuron-specific markers with protein expression highly reminiscent to the genetically matched control
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 VAcHT and VAChT in ioMotor Neurons FUS P525L/WT compared to the genetically matched control, ioMotor Neurons.
ioMotor Neurons FUS P525L/WT demonstrate gene expression of neuronal-specific and motor neuron-specific markers following deterministic programming
Gene expression analysis demonstrates that ioMotor Neurons FUS P525L/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), 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.
Disease-related FUS is expressed in ioMotor Neurons FUS P525L/WT following deterministic programming
Gene expression analysis demonstrates that ioMotor Neurons FUS P525L/WT and the genetically matched control (WT) express the FUS gene encoding the Fused in Sarcoma protein. 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.
Do more with every vial
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.
ioMotor Neurons FUS P525L/WT form a homogenous neuronal network by day 4
ioMotor Neurons FUS P525L/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.
ioMotor Neurons FUS P525L/WT express motor neuron-specific markers with protein expression highly reminiscent to the genetically matched control
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 VAcHT and VAChT in ioMotor Neurons FUS P525L/WT compared to the genetically matched control, ioMotor Neurons.
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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).
Access 14 ALS and FTD disease models with disease-related mutations such as SOD1, FUS, MAPT and TDP-43 (TARDBP) genetically engineered in ioGlutamatergic Neurons and ioMotor Neurons.
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!
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
ioMotor Neurons
ioAstrocytes
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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Consistent. Defined. Scalable.
