Hero-PINK1-Q456X-Hom_ICC_DAPI_MAP2

cat no | io1075, io1076

ioGlutamatergic Neurons PINK1 Q456X/Q456X

Human iPSC-derived Parkinson's disease model

ioGlutamatergic Neurons PINK1 Q456X/Q456X are opti‑ox deterministically programmed glutamatergic neurons carrying a genetically engineered homozygous 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).

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.

per vial

Benchtop benefits

comparison_0

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_0

Scalable

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

quick_0

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.

Technical data

Highly characterised and defined

ioGlutamatergic Neurons PINK1 Q456X/Q456X express neuron-specific markers comparably to the wild type control
io1075-io1076-ioGlutamatergic Neurons PINK1-Q456X-hom-ICC-MAP2-TUBB3
io1075-io1076-ioGlutamatergic Neurons PINK1-Q456X-hom-ICC-MAP2-VGLUT2
Immunofluorescent staining on post-revival day 11 demonstrates similar homogenous expression of pan-neuronal proteins MAP2 and TUBB3 (left tab) and glutamatergic neuron-specific transporter VGLUT2 (right tab) in ioGlutamatergic Neurons PINK1 Q456X/Q456X clones compared to the genetically matched control. 100X magnification.
ioGlutamatergic Neurons PINK1 Q456X/Q456X form structural neuronal networks by day 11
io1075-io1076-ioGlutamatergic Neurons PINK1-Q456X-hom-Morphology
ioGlutamatergic Neurons PINK1 Q456X/Q456X clones mature rapidly and form structural neuronal networks over 11 days, highly similar to the genetically matched control. Day 1 to 11 post thaw; 100X magnification.
ioGlutamatergic Neurons PINK1 Q456X/Q456X demonstrate gene expression of neuronal-specific and glutamatergic-specific markers following deterministic programming
io1075-io1076_PINK-Q456X-Hom_RT-qPCR
Gene expression analysis demonstrates that ioGlutamatergic Neurons PINK1 Q456X/Q456X clones and wild-type ioGlutamatergic Neurons (WT Control) lack the expression of pluripotency markers (NANOG and OCT4) at day 11, while expressing pan-neuronal (TUBB3 and SYP) and glutamatergic-specific (VGLUT1 and VGLUT2) markers, as well as the glutamate receptor GRIA4. Gene expression levels were assessed by RT-qPCR (data normalised to HMBS; cDNA samples of the parental human iPSC line (hiPSC) were included as reference). Data represents day 11 post-revival samples, n=2 replicates.
Disease-related PINK1 is expressed in ioGlutamatergic Neurons PINK1 Q456X/Q456X following deterministic programming
io1075-io1076_PINK-Q456X-Hom-PINK1-RT-qPCR
RT-qPCR analysis demonstrates expression of the PINK1 gene in both wild type ioGlutamatergic Neurons (WT Control) and ioGlutamatergic Neurons PINK1 Q456X/Q456X clones at day 11 post-revival. Data normalised to HMBS, n=2 replicates.

Cells arrive ready to plate

ioGlutamatergic_Neurons_and_disease_models_timeline
ioGlutamatergic Neurons PINK1 Q456X/Q456X are delivered in a cryopreserved format and are programmed to mature rapidly upon revival in the recommended media. The protocol for the generation of these cells is a two-phase process: Phase 1, Stabilisation for 4 days; Phase 2, Maintenance, during which the neurons mature. Phases 1 and 2 after revival of cells are carried out by the customer.

Industry leading seeding density

ioGlutamatergic_Neurons_seeding_density_small_96_384
The recommended minimum seeding density is 30,000 cells/cm2, compared to up to 250,000 cells/cm2 for other similar products on the market. One small vial can plate a minimum of 0.7 x 24-well plate, 1 x 96-well plate, or 1.5 x 384-well plates. This means every vial goes further, enabling more experimental conditions and more repeats, resulting in more confidence in the data.

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 deterministic cell programming

Recommended seeding density

30,000 cells/cm²

User storage

LN2 or -150°C

Format

Cryopreserved cells

Genetic modification

Homozygous Q456X nonsense mutation in the PINK1 gene

Applications

Parkinson's disease research
Drug discovery and development
Disease modelling

Available clones

io1075 | PINK1 Q456X/Q456X (CL49)
io1076 | PINK1 Q456X/Q456X (CL77)

Product use

ioCells are for research use only

Product resources

ioGlutamatergic Neurons Brochure
ioGlutamatergic Neurons

bit.bio

Download
ioGlutamatergic Neurons Wild Type and related disease models | User Manual User manual
ioGlutamatergic Neurons Wild Type and related disease models | User Manual

V11

bit.bio

2024

Download
Generation and characterisation of a panel of human iPSC-derived neurons and microglia carrying early and late onset relevant mutations for Alzheimer’s disease 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
Download
Generating publishable neuroscience research in 12 weeks with ioGlutamatergic Neurons 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 

Download
Running Large-Scale CRISPR Screens in Human Neurons 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
Addressing the Reproducibility Crisis | Driving Genome-Wide Consistency in Cellular Reprogramming 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
Industrialising Cellular Reprogramming: Leveraging opti-ox Technology to Manufacture Human Cells with Unprecedented Consistency 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
Modelling neurodegeneration: Human isogenic system to study FTD & ALS Poster
Modelling neurodegeneration: Human isogenic system to study FTD & ALS

Oosterveen, et al

bit.bio & Charles River Laboratories

2023

View
Rethinking Developmental Biology With Cellular Reprogramming 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
Precision Cellular Reprogramming for Scalable and Consistent Human Neurodegenerative Disease Models Talk
Precision Cellular Reprogramming for Scalable and Consistent Human Neurodegenerative Disease Models

Madeleine Garrett | Field Application Specialist | bit.bio

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

Ritsma et al

Charles River Laboratories & bit.bio

2022

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

Raman, et al

bit.bio

2022

View
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 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

Download
Interferon-γ exposure of human iPSC-derived neurons alters major histocompatibility complex I and synapsin protein expression Publication
Interferon-γ exposure of human iPSC-derived neurons alters major histocompatibility complex I and synapsin protein expression

Pavinlek, et al

Frontiers in Psychiatry

2022

 

Using ioGlutamatergic Neurons

 

 

Read more
Glutamatergic Neurons and Brain Cyst Formation Publication
Glutamatergic Neurons and Brain Cyst Formation

Bando, et al

Frontiers in Cellular and Infection Microbiology

2022

 

Using ioGlutamatergic Neurons

 

 

Read more
Compounds co-targeting kinases in axon regulatory pathways promote regeneration and behavioral recovery after spinal cord injury in mice Publication
Compounds co-targeting kinases in axon regulatory pathways promote regeneration and behavioral recovery after spinal cord injury in mice

Mah, et al

Experimental Neurology

2022

 

Using ioGlutamatergic Neurons

Read more

Cell culture hacks | human iPSC-derived glutamatergic neurons

Read this blog on glutamatergic neuron cell culture for our top tips on careful handling, cell plating and media changes to achieve success from the outset.

bit.bio_3x2_ioGlutamatergic Neurons_MAP2_Hoescht_x20_hi.res (1)

Related products

ioGlutamatergic Neurons PINK1 Q456X/WT ioDisease Model Cells
ioGlutamatergic Neurons PINK1 Q456X/WT cat no. io1078 | io1079 | io1080
Order now
ioGlutamatergic Neurons PRKN R275W/WT ioDisease Model Cells
ioGlutamatergic Neurons PRKN R275W/WT cat no. io1013
Order now
ioGlutamatergic Neurons SNCA A53T/A53T ioDisease Model Cells
ioGlutamatergic Neurons SNCA A53T/A53T cat no. io1087 | io1088 | io1089
Order now
ioGlutamatergic Neurons PRKN R275W/R275W ioDisease Model Cells
ioGlutamatergic Neurons PRKN R275W/R275W cat no. io1020
Order now
CRISPR-Ready ioGlutamatergic Neurons CRISPR-Ready ioCells
CRISPR-Ready ioGlutamatergic Neurons cat no. io1090S
Order now
ioGlutamatergic Neurons ioWild Type Cells
ioGlutamatergic Neurons cat no. io1001
Order now

Wild Type and Isogenic Disease Model cells: A true comparison

Further your disease research by pairing our wild type cells with isogenic disease models.

bitbio-vials-Wild_and_Disease-staggered-2500px_wide

Related pages

Discover ioCells Learn about our range of human iPSC-derived cells for research and drug discovery
Resources Explore our latest scientific insights, webinars, blogs and videos
Our platform Discover the cell identity coding platform that powers our ioCells