ioGlutamatergic Neurons TDP-43 M337V homozygous - Human iPSC derived cells available to order. Wild type ioGlutamatergic Neurons (ab303447) form the genetically matched control for the ioGlutamatergic Neurons TDP-43 M337V homozygous disease model.
ioGlutamatergic Neurons TDP-43 M337V homozygous - Human iPSC derived cells available to order. Wild type ioGlutamatergic Neurons (ab303447) form the genetically matched control for the ioGlutamatergic Neurons TDP-43 M337V homozygous disease model.
Wild type ioGlutamatergic Neurons (ioGlutamatergic Neurons WT (isogenic control) - Human iPSC derived cells ab303447) form the genetically matched control for the ioGlutamatergic Neurons TDP-43 M337V homozygous disease model. This physiologically-relevant isogenic pairing offers a powerful next generation model to study amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) in research and drug discovery.
ioGlutamatergic Neurons TDP-43 M337V homozygous are ioGlutamatergic Neurons carrying a genetically engineered M337V mutation in the TARDBP gene, encoding TAR DNA binding protein 43 (TDP-43). ioGlutamatergic Neurons TDP-43 M337V homozygous have been reprogrammed from human iPSCs using opti-oxTM technology, a precise reprogramming technology.
Human stem cells, within days, convert consistently into mature, functional glutamatergic neurons providing a high quality human model for the study of ALS and FTD.
ioGlutamatergic Neurons TDP-43 M337V homozygous express pan-neuronal and glutamatergic markers TUBB3, MAP2 and VGLUT2 by day 11.
This disease model offers a fast and easy-to-use system for investigations into the impact of gene function on disease progression against an isogenic control.
A heterozygous disease model is also available (ioGlutamatergic Neurons TDP-43 M337V heterozygous - Human iPSC derived cells ab307782).
In partnership with bit.bio
Karyotype: Normal
Seeding Density: 30,000 cells/cm2
Seeding compatibility: 6-, 12-, 24-, 96- and 384-well compatible
Quality control: ICC and gene expression analysis
Research applications: Academic research, Drug development, Neurotoxicology, Genetic screening (e.g. CRISPR screening).
This product is subject to limited use licenses from iPS Academia Japan Inc, TET Systems GmbH, ERS Genomics Limited and Sigma-Aldrich Co. LLC and is developed with Bit Bio patented technology. For full details of the licenses and patents please refer to our limited use license and patent pages.
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ioGlutamatergic Neurons TDP-43M337V/WT and ioGlutamatergic Neurons TDP-43M337V/M337V mature rapidly and form structural neuronal networks over 11 days when compared to the isogenic control. Day 1 to 11 post-thawing; 100X magnification.
Gene expression analysis demonstrates that ioGlutamatergic Neurons TDP-43M337V/WT, ioGlutamatergic Neurons TDP-43M337V/M337V and the isogenic control (WT) lack the expression of pluripotency makers (NANOG and OCT4), at day 11, whilst robustly expressing pan-neuronal (TUBB3 and SYP) and glutamatergic-specific (VGLUT1 and VGLUT2) markers, and the glutamate receptor GRIA4. Gene expression levels were assessed by RT-qPCR (data expressed relative to the parental hiPSC control (iPSC Control), normalised to HMBS). Data represents day 11 post-revival samples.
Gene expression analysis demonstrates that ioGlutamatergic Neurons TDP-43M337V/WT, ioGlutamatergic Neurons TDP-43M337V/M337V and the isogenic control (WT) express the TARDBP gene encoding TDP-43. Gene expression levels were assessed by RT-qPCR (data expressed relative to the parental hiPSC control (iPSC Control), normalised to HMBS). Data represents day 11 post-revival samples.
Immunofluorescent staining on post-revival day 11 demonstrates similar homogenous expression of glutamatergic neuron-specific transporter VGLUT2 (upper panel) and pan-neuronal proteins MAP2 and TUBB3 (lower panel) in ioGlutamatergic Neurons TDP-43M337V/WT and ioGlutamatergic Neurons TDP-43M337V/M337V compared to the isogenic control. 100X magnification.
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