cat no | io1052
ioMotor Neurons FUS P525L/P525L
Human iPSC-derived ALS disease model
ioMotor Neurons FUS P525L/P525L are opti‑ox deterministically programmed ioMotor Neurons carrying a genetically engineered homozygous 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 homozygous P525L mutation in ALS.
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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.
Benchtop benefits
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.
Ready within days
Schematic overview of the timeline in the user manual
ioMotor Neurons FUS P525L/P525L are delivered in a cryopreserved format and are programmed to rapidly mature upon revival in the recommended media.
ioMotor Neurons FUS P525L/P525L form a homogenous neuronal network by day 4
ioMotor Neurons FUS P525L/P525L 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/P525L express motor neuron-specific markers with protein expression highly reminiscent to the genetically matched control
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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/P525L compared to the genetically matched control, ioMotor Neurons.
ioMotor Neurons FUS P525L/P525L demonstrate gene expression of neuronal-specific and motor neuron-specific markers following deterministic programming
Gene expression analysis demonstrates that ioMotor Neurons FUS P525L/P525L 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/P525L 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
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.
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).
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