ioGABA APP V7171 Hom Hero image compressed 2

cat no | io1081, io1082

ioGABAergic Neurons APP V717I/V717I^™

Human iPSC-derived Alzheimer's disease model

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.

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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.

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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.

Highly characterised and defined

Disease model cells express key GABAergic neuron-specific markers comparably to the isogenic control

ioGABA APP V717I Hom ICC panel FINAL compressed

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

bit.bio_ioGABAergic Neurons_timeline_horizontal_withoutdox

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.

Product information

Starting material

Human iPSC line

Karyotype

Normal (46, XY)

Seeding compatibility

6, 12, 24, 96 and 384 well plates

Shipping info

Dry ice

Donor

Caucasian adult male (skin fibroblast)

Vial size

Small: >3 x 10⁶ viable cells

Quality control

Sterility, protein expression (ICC) and gene expression (RT-qPCR)

Differentiation method

opti-ox cellular reprogramming

Recommended seeding density

150,000 cells/cm²

User storage

LN2 or -150°C

Format

Cryopreserved cells

Product use

ioCells are for research use only

Genetic modification

Homozygous V717I mutation in the APP gene

Applications

Alzheimer's disease modelling
Drug discovery and development
MEA analysis
Co-culture studies
ASO screening

Available clones

io1081S: ioGABAergic Neurons APP V717I/V717I (CL59)
io1082S: ioGABAergic Neurons APP V717I/V717I (CL70)

Supporting documentation

User Manual

Limited Use License & Statement of Use

Product resources

Poster

Generation and characterisation of a panel of human iPSC-derived neurons and microglia carrying early and late onset relevant mutations for Alzheimer’s disease

Smith et al.
bit.bio
2024

Downlaod

Webinar

Addressing the Reproducibility Crisis | Driving Genome-Wide Consistency in Cellular Reprogramming

Dr Ania Wilczynska | Head of Computational Genomics | Non-Clinical | bit.bio

Watch now

Talk

Industrialising Cellular Reprogramming: Leveraging opti-ox™ Technology to Manufacture Human Cells with Unprecedented Consistency

Innovation showcase talk at ISSCR

Marius Wernig MD, PhD | Stanford

Mark Kotter, MD, PhD | bit.bio

Watch now

Webinar

Mastering Cell Identity In A Dish: The Power Of Cellular Reprogramming

Prof Roger Pedersen | Adjunct Professor and Senior Research Scientist at Stanford University

Dr Thomas Moreau | Director of Cell Biology Research | bit.bio

Watch now

Webinar

Rethinking Developmental Biology With Cellular Reprogramming

Mark Kotter | CEO and founder | bit.bio

Marius Wernig | Professor Departments of Pathology and Chemical and Systems Biology | Stanford University

Watch now

Infographic

Comparing Stem Cells Differentiation Methods | Infographic

bit.bio

Download

Webinar

Modelling neurodevelopment | Investigating the impact of maternal immune activation on neurodevelopment using human iPSC-derived cells

Dr Deepak Srivastava | King’s College London

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Webinar

Modelling human neurodegenerative diseases in research & drug discovery

Dr Mariangela Iovino | Group Leader | Charles River

Dr Tony Oosterveen | Senior Scientist | bit.bio

Watch now

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).

Watch the webinar