cat no | io1041S
ioMotor Neurons SOD1 G93A/G93A are opti‑ox deterministically programmed ioMotor Neurons carrying a genetically engineered homozygous mutation in the SOD1 gene encoding the Superoxide dismutase 1 protein.
Within days, cells convert to a defined and scalable genetically matched system for investigating the molecular and cellular significance of a homozygous G93A mutation in ALS.
Related disease model cells are available with a heterozygous SOD1 G93A/WT mutation, and both can be used alongside their genetically matched control, ioMotor Neurons.
Additional disease models are available in ioGlutamatergic Neurons with mutations in TDP‑43 and MAPT, creating a comprehensive toolkit to study the genetic and pathological overlap between ALS and FTD.
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
A maximum number of 20 vials applies. If you would like to order more than 20 vials, please contact us at orders@bit.bio.
Make True Comparisons
Pair the ioDisease Model Cells with genetically matched wild-type ioMotor Neurons to investigate the impact of mutant SOD1 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.
Schematic overview of the timeline in the user manual
ioMotor Neurons SOD1 G93A/G93A are delivered in a cryopreserved format and are programmed to rapidly mature upon revival in the recommended media.
ioMotor Neurons SOD1 G93A/G93A form a homogenous neuronal network by day 4
ioMotor Neurons SOD1 G93A/G93A 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 SOD1 G93A/G93A compared to the genetically matched control, ioMotor Neurons.
Gene expression analysis demonstrates that ioMotor Neurons SOD1 G93A/G93A 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.
Gene expression analysis demonstrates that ioMotor Neurons SOD1 G93A/G93A and the genetically matched control (WT) express the SOD1 gene encoding the Superoxide dismutase 1 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
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
Luke Foulser | Scientist | bit.bio
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).