cat no | io1078, io1079, io1080

ioGlutamatergic Neurons PINK1 Q456X/WT^™

Human iPSC-derived Parkinson's disease model

ioGlutamatergic Neurons PINK1 Q456X/WT are opti‑ox™ precision reprogrammed glutamatergic neurons carrying a genetically engineered heterozygous Q456X mutation in the PINK1 gene encoding the protein PTEN induced putative kinase 1. These cells offer a rapidly maturing, disease relevant system for investigating the role of PINK1 Q456X mutation in early-onset Parkinson's disease (PD).

This disease model is part of a Parkinson's disease panel of physiologically relevant human iPSC-derived cells that can be incorporated into translational research and drug discovery workflows. Additional mutations include homozygous PINK1 Q456X mutation, PRKN R275W, SNCA A53T and GBA mutations. All can be used alongside their genetically matched control, ioGlutamatergic Neurons™.

Coming Soon

Benchtop benefits

Make True Comparisons

Pair the ioDisease Model Cells with the genetically matched wild-type ioGlutamatergic Neurons to investigate the impact of the PINK1 nonsense mutation on early-onset PD.

Scalable

With opti-ox technology, we can make billions of consistently reprogrammed cells, surpassing the demands of industrial workflows.

Quick

The disease model cells and isogenic control are experiment ready as early as 2 days post revival, and form structural neuronal networks at 11 days.

Product information

Starting material

Human iPSC line

Karyotype

Normal (46, XY)

Seeding compatibility

6, 12, 24, 96 & 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), gene expression (RT-qPCR) and genotype validation (Sanger sequencing)

Differentiation method

opti-ox cellular reprogramming

Recommended seeding density

30,000 cells/cm²

User storage

LN2 or -150°C

Format

Cryopreserved cells

Genetic modification

Heterozygous Q456X nonsense mutation in the PINK1 gene

Applications

Parkinson's disease research
Drug discovery and development
Disease modelling

Available clones

io1078 | PINK1 Q456X/WT (CL1)
io1079 | PINK1 Q456X/WT (CL187)
io1080 | PINK1 Q456X/WT (CL44)

Product use

ioCells are for research use only

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

User manual

ioGlutamatergic Neurons Wild Type and related disease models | User Manual

bit.bio

2024

Download

Case study

Generating publishable neuroscience research in 12 weeks with ioGlutamatergic Neurons™

Professor Deepak Srivastava

Professor of Molecular Neuroscience and Group Leader, MRC Centre for Developmental Disorders

King’s College London

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Webinar

Running Large-Scale CRISPR Screens in Human Neurons

Emmanouil Metzakopian | Vice President, Research and Development | bit.bio

Javier Conde-Vancells | Director Product Management | bit.bio

Watch now

Publication

3D bioprinting of iPSC neuron-astrocyte coculture

Whitehouse, et al
JoVE Journal of Visualized Experiments
2023

Using ioGlutamatergic Neurons

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

Poster

Modelling neurodegeneration: Human isogenic system to study FTD & ALS

Oosterveen, et al

bit.bio & Charles River Laboratories

2023

Download

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

Talk

Precision Cellular Reprogramming for Scalable and Consistent Human Neurodegenerative Disease Models

Madeleine Garrett | Field Application Specialist | bit.bio

Watch now

Poster

Development and characterisation of a robust in vitro disease model to study tauopathies

Ritsma et al

Charles River Laboratories & bit.bio

2022

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Poster

Validation of ALS-relevant phenotypes in precision reprogrammed iPSC-derived glutamatergic Neurons containing a TDP-43 M337V mutation.

Ritsma, et al

Charles River Laboratories & bit.bio

2022

Download

Poster

Rapid and consistent generation of functional microglia from reprogrammed hiPSCs to study neurodegeneration and neuroinflammation

Raman, et al

bit.bio

2022

Download

Application note

Developing next-generation in vitro phenotypic assays for Huntington’s disease by combining a precision reprogrammed hiPSC-derived disease model with high-density microelectrode arrays

bit.bio | MaxWell Biosystems | Charles River Laboratories

2022

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