ioMotor DM HERO RECT

cat no | io1055S

ioMotor Neurons FUS P525L/WT

Human iPSC-derived ALS disease model

ioMotor Neurons FUS P525L/WT are opti‑ox deterministically programmed ioMotor Neurons carrying a genetically engineered heterozygous mutation in the FUS gene encoding the Fused in Sarcoma protein. 

Within days, cells convert to a defined and scalable genetically matched system for investigating the molecular and cellular significance of a heterozygous P525L mutation in ALS.

Place your order

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.

per vial

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 FUS 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.

Technical data

Ready within days

Schematic overview of the timeline in the user manual

ioMotor Neuron + DM timeline-1

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

Brightfield image - ioMotor_FUS_P525L HET
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.

Highly characterised and defined

ioMotor Neurons FUS P525L/WT express motor neuron-specific markers with protein expression highly reminiscent to the genetically matched control

ICC Panel 1 - ioMotor_FUS-P525L HET
ICC Panel 2 - ioMotor_FUS-P525L HET

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
FUS HET

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
FUSHET GE

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.

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.

Product information

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 disease modelling
Electrophysiological analysis
Drug development & discovery
Neuromuscular research
Neurotoxicology

Product resources

ioMotor Neurons and related disease models | User Manual User manual
ioMotor Neurons and related disease models | User Manual
V5
2024
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
ioMotor Neurons Brochure
ioMotor Neurons
bit.bio
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

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Related pages

Discover ioCells Learn about our range of human iPSC-derived cells for research and drug discovery
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