ioGABAergic Neurons APP V717I/V717I are opti-ox™ precision reprogrammed 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.
Two clones are available, all genetically matched to the wild type control (ioGABAergic Neurons™). The disease model cells and the wild-type control offer a physiologically relevant model to investigate the impact of the APP V717I mutation on cellular and molecular mechanisms and function in early-onset AD.
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.
A maximum number of 20 vials applies. If you would like to order more than 20 vials, please contact us at firstname.lastname@example.org.
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 reprogramming
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 bit.bio, 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.
Human iPSC line
Normal (46, XY)
6, 12, 24, 96 and 384 well plates
Caucasian adult male (skin fibroblast)
Small: >3 x 10⁶ viable cells
Sterility, protein expression (ICC) and gene expression (RT-qPCR)
opti-ox cellular reprogramming
Recommended seeding density
LN2 or -150°C
ioCells are for research use only
Homozygous V717I mutation in the APP gene
Alzheimer's disease modelling Drug discovery and development MEA analysis Co-culture studies ASO screening
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).