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.
ioGlutamatergic Neurons GBA null/R159W express neuron-specific markers comparably to the isogenic control
ioGlutamatergic Neurons GBA null/R159W form structural neuronal networks by day 11
ioGlutamatergic Neurons GBA null/R159W demonstrate gene expression of neuronal and glutamatergic-specific markers following deterministic programming
Disease-related GBA is expressed in ioGlutamatergic Neurons GBA null/R159W following deterministic programming
GBA protein is present in ioGlutamatergic Neurons GBA null/R159W at a lower level than the wild type control
*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
Chakraborty et al
Nature Communications
2023
Featuring ioGlutamatergic Neurons
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
Qiaojin Lin et al
The EMBO Journal
2023
Featuring opti-ox powered hiPSC-derived glutamatergic neurons with constitutive expression of Cas9
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
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.