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iNeu™ Glutamatergic Neurons TDP-43 M337V/WT, ALS Model

[CAT#: NRZP-0323-ZP8]

Human iPSC-derived ALS and FTD disease model

Species:
Human
Cell Types:
Glutamatergic Neurons

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Product Overview

Description

iNeu™ Glutamatergic Neurons TDP-43 M337V/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 mutation in the TARDBP gene, encoding TAR DNA-binding protein 43 (TDP-43). 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.

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

Mutation Description

Heterozygous M337V missense mutation in the TARDBP gene

Relevant Diseases

FTD; ALS

Species

Human
Properties

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)

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Related Products
For Research Use Only. Not For Clinical Use.
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