cat no | io1007
ioGlutamatergic Neurons GBA null/R159W are opti‑ox deterministically programmed glutamatergic neurons carrying a genetically engineered compound heterozygous mutation in the GBA gene encoding the glucocerebrosidase (GCase) enzyme. These cells offer a rapidly maturing, human cell model to investigate modulation of GCase expression.
Related Parkinson's disease model cells are available with PINK1 Q456X, PRKN R275W and SNCA A53T mutations, and all can be used alongside their genetically matched control, ioGlutamatergic Neurons.
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
A maximum number of 20 vials applies. If you would like to order more than 20 vials, please contact us at orders@bit.bio.
Make True Comparisons
Pair the ioDisease Model Cells with the genetically matched wild-type ioGlutamatergic Neurons to investigate the impact of the GBA mutation on molecular mechanisms and cell function.
Scalable
With opti-ox technology, we can make billions of consistently programmed 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.
A Western blot experiment confirmed the presence of the GBA protein in ioGlutamatergic Neurons GBA null/R159W at a lower level than in the wild-type ioGlutamatergic Neurons. Day 11 cell lysates were subjected to Western blotting (20 µg protein in 40 µl per lane) using 4-20% mini protean TGX stain-free gels. Proteins were transferred onto PVDF membranes using the Trans-Blot Turbo Transfer Pack, blocked for 10 minutes, incubated with primary antibodies (GBA Invitrogen MA5-26589, 1:2000; GAPDH Abcam ab8245, 1:5000), washed three times, incubated with HRP-labelled secondary antibodies, washed three times and signal visualised by electrochemiluminescence.
1= ioGlutamatergic Neurons (wild type), 2= ioGlutamatergic Neurons GBA null/R159W.
Starting material
Human iPSC line
Karyotype
Normal (46, XY)
Seeding compatibility
6, 12, 24, 48, 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 (long amplicon 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
Compound heterozygous null/R159W mutation in the GBA gene*
Applications
Gaucher and Parkinson's disease research
Drug discovery and development
Disease modelling
Product use
ioCells are for research use only
*The null allele has a 1 base heterozygous deletion at position chr1:155,238,622 (GRCh38) located in coding exon 6, causing a frameshift resulting in a series of STOP codons (ENST00000574670.5). The second allele has a missense mutation, R159W (NM_000157.4(GBA1):c.475C>T (p.Arg159Trp))
bit.bio
V11
bit.bio
2024
Professor Deepak Srivastava
Professor of Molecular Neuroscience and Group Leader, MRC Centre for Developmental Disorders
King’s College London
Emmanouil Metzakopian | Vice President, Research and Development | bit.bio
Javier Conde-Vancells | Director Product Management | bit.bio
Dr Ania Wilczynska | Head of Computational Genomics | Non-Clinical | bit.bio
Innovation showcase talk at ISSCR
Marius Wernig MD, PhD | Stanford
Mark Kotter, MD, PhD | bit.bio
Oosterveen, et al
bit.bio & Charles River Laboratories
2023
Mark Kotter | CEO and founder | bit.bio
Marius Wernig | Professor Departments of Pathology and Chemical and Systems Biology | Stanford University
Madeleine Garrett | Field Application Specialist | bit.bio
Ritsma, et al
Charles River Laboratories & bit.bio
2022
Raman, et al
bit.bio
2022
bit.bio | MaxWell Biosystems | Charles River Laboratories
2022
Pavinlek, et al
Frontiers in Psychiatry
2022
Using ioGlutamatergic Neurons
Bando, et al
Frontiers in Cellular and Infection Microbiology
2022
Using ioGlutamatergic Neurons
Mah, et al
Experimental Neurology
2022
Using ioGlutamatergic Neurons
Dr Deepak Srivastava | King’s College London
Dr Marijn Vlaming | Head of Biology, et al.
Charles River & bit.bio
Dr Kaiser Karim | Scientist
bit.bio
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.
Further your disease research by pairing our wild type cells with isogenic disease models.