cat no | io1081, io1082
ioGABAergic Neurons APP V717I/V717I
Human iPSC-derived Alzheimer's disease model
ioGABAergic Neurons APP V717I/V717I are opti-ox deterministically programmed GABAergic neurons carrying a genetically engineered homozygous V7171 (London) mutation in the APP gene encoding the amyloid precursor protein. This mutation is linked to familial early-onset Alzheimer's disease (AD).
These cells offer a functional, rapidly maturing, and disease relevant system to study the role of the APP V717I (London) mutation in early-onset AD, alongside a genetically matched wild-type control.
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.
Benchtop benefits
Make True Comparisons
Pair the ioDisease Model Cells with the genetically matched wild-type ioGABAergic Neurons to directly investigate the effect of the mutant APP protein on early-onset AD.
Highly pure
>99% of cells express key GABAergic markers within 4 days post-thaw, allowing consistent and reproducible results from every vial.
Co-culture compatible
Suitable for co-culture and tri-culture studies with ioGlutamatergic Neurons and astrocytes.
Disease related phenotype
Increased ratio of A饾浗42:40 seen in ioGABAergic Neurons APP V717I (London), as observed in Alzheimer鈥檚 disease
ioGABAergic Neurons APP V717I/V717I disease model (HOM) cells show a trend of increased production of A饾浗38 peptides and A饾浗42 (involved in the amyloidogenic pathway), with minimal difference seen for A饾浗40 (A). This results in a significantly increased ratio of A饾浗42:40 and no change in the A饾浗42:38 ratio (B).
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ioGABAergic neurons (WT, CL54, CL65, CL59, CL70) were seeded at 150,000 cells/cm2 in 24 well plates and cultured for 30 days according to the user manual.
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Supernatant was collected at days 10, 20, and 30 to quantify levels of A饾浗38, A饾浗40, A饾浗42 peptides using the V-PLEX A尾 Peptide Panel ELISA kit (MSD K15200E-1).
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Concentrations of A饾浗38, A饾浗40, A饾浗42 were normalised to the calculated total number of cells per well.
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Data were obtained from two independent experiments and are shown as mean 卤 SEM. Data were analysed statistically (at days 20 and 30) using one-way ANOVA with Tukey鈥檚 post-hoc analysis. * p<0.05 ** p<0.01 ***p<0.001 **** p<0.0001
Highly characterised and defined
Disease model cells express key GABAergic neuron-specific markers comparably to the isogenic control
Immunofluorescent staining on day 12 post-revival demonstrates similar homogenous expression of the pan-neuronal marker, MAP2 and GABAergic neuron-specific marker, GABA in both disease model clones compared to the wild-type (WT) isogenic control. 100X magnification.
Disease model cells form structural neuronal networks by day 12
Both disease model clones mature rapidly and form structural neuronal networks over 12 days, with neurons identified by day 3 and visible neuronal networks being observed by day 10 post-thaw, similarly to the WT isogenic control. Day 1 to 12 post thawing; 100X magnification.
Disease model cells demonstrate gene expression of neuronal and GABAergic-specific markers following deterministic programming
Gene expression analysis demonstrates that both disease model clones and the WT isogenic control lack the expression of pluripotency markers (NANOG and OCT4) at day 12 post-thaw, whilst robustly expressing pan-neuronal (TUBB3) and GABAergic-specific markers, GAD1, GAD2, VGAT, DLX1, and DLX2. Gene expression levels were assessed by RT-qPCR (data expressed relative to the parental hiPSC control (iPSC Control), normalised to GAPDH). Data represents day 12 post-revival samples.
Cells arrive ready to plate
ioGABAergic Neurons APP V717I/V717I are delivered in a cryopreserved format and are programmed to rapidly mature upon revival in the recommended media. The protocol for the generation of these cells is a two-phase process: Induction, which is carried out at 樱桃视频, Stabilisation for 3 days (Phase 1), and Maintenance (Phase 2) during which the ioGABAergic Neurons mature. Phases 1 and 2 after revival of cells are carried out at the customer site.
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The Potential of RNA Therapies in Autism Spectrum Disorder
In this webinar, Dr Rodney Bowling, CSO of Everlum Bio, offers an expert discussion on their use of ioGABAergic neurons for the screening of antisense oligonucleotide (ASO) based RNA therapeutics to accelerate the discovery of novel personalised therapies for rare autism spectrum disorders (ASD).
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