iNeu™ Glutamatergic Neurons TDP-43 M337V/M337V, ALS Model
[CAT#: NRZP-0323-ZP9]
Human iPSC-derived ALS and FTD disease model
- Species:
- Human
- Cell Types:
- Glutamatergic Neurons
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To download a Certificate of Analysis, please enter a lot number in the search box below. Note: Certificate of Analysis not available for kit components.
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Product Overview
Description
iNeu™ Glutamatergic Neuron TDP-43 WT is a rapidly maturing, consistent and scalable isogenic system for the study of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). They are precisely reprogrammed glutamatergic neurons carrying a genetically engineered heterozygous M337V mutant gene encoding TAR DNA-binding protein 43 (TDP-43) in TARDBP.
Relevant disease model cells have homozygous TDP-43 M337V/M337V mutations, and both can be used with their genetically matched controls.
Relevant disease model cells have homozygous TDP-43 M337V/M337V mutations, and both can be used with their genetically matched controls.
Features
Making real comparisons
Disease model cells were paired with genetically matched wild-type glutamatergic neurons to study the effects of mutant TDP-43 proteins on disease progression.
Scalable
Available in industrial-scale quantities, with industry-leading seeding densities, and at an affordable price, cells can be used from research to high-throughput screening.
Fast
Disease model cells and isogenic controls were ready for experiments as early as 2 days after resuscitation and formed structural neuronal networks by 11 days.
Disease model cells were paired with genetically matched wild-type glutamatergic neurons to study the effects of mutant TDP-43 proteins on disease progression.
Scalable
Available in industrial-scale quantities, with industry-leading seeding densities, and at an affordable price, cells can be used from research to high-throughput screening.
Fast
Disease model cells and isogenic controls were ready for experiments as early as 2 days after resuscitation and formed structural neuronal networks by 11 days.
Neurological Disease Models
ALS-related Cells
Cell Types
Glutamatergic Neurons
Application Notes
FTD and ALS research
Drug discovery and development
Disease modelling
High-throughput screening
Electrophysiological assays (MEA)
Co-culture studies
Drug discovery and development
Disease modelling
High-throughput screening
Electrophysiological assays (MEA)
Co-culture studies
Mutation Description
Homozygous M337V missense mutation in the TARDBP gene
Relevant Diseases
FTD; ALS
Species
Human
Properties
Size
>1 x 10^6 viable cells
Form
Frozen
Cell State
Cryopreserved cells
Growth Pattern
Adherent
Seeding Density
6, 12, 24, 48, 96 & 384 well plates
Tissue Source
Skin fibroblast
Shipping
Dry ice
Storage
LN2 or -150°C
Quality Control
Sterility, protein expression (ICC), gene expression (RT-qPCR) and genotype validation
Donor Information
Gender
Male
Donor Age
Adult
Ethnicity
Caucasian
Additional Donor Information
Normal (46, XY)
Publications
Publications (0)
Neural Cell Lines
For Research Use Only. Not For Clinical Use.
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