Creative Biolabs

iNeu Motor Neurons ALS TDP43 Q331K, Disease Model

[CAT#: NCL-7P001]

Motor Neurons ALS/FTD differentiated from Human iPS cells, frozen

Species:
Human
Applications:
Cell Assay
Cell Types:
Motor Neuron

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Description
Tar DNA binding protein (TDP-43) is a highly conserved ribonucleoprotein that accumulates as ubiquitinated inclusions in patients with amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). TDP-43 usually shuttles between the nucleus and cytoplasm, and plays a role in regulating RNA splicing, stability and transport. TDP-43 neuropathology is present in most familial and sporadic ALS; therefore, understanding TDP-43 dysfunction may provide a way for therapeutic intervention. Further TDP-43 mutations, including M337V and Q331K variants, account for nearly 5% of familial ALS.

In order to promote ALS/FTD research work, an isogenic model of ALS motor neuron was generated. Using CRISPR gene editing to introduce M337V or Q331K mutations into our iNeu motor neuron parental induced pluripotent stem (iPS) cell line. These gene-edited iPS cells are fully differentiated into high-purity Human cells, showing similar indicators to our apparently healthy iNeu motor neurons.

The isogenic disease model iNeu motor neuron ALS TDP43 M337V and TDP43 Q331K combined with iNeu motor neuron provides a precision medicine-based method that can promote functional genomics research, identification of drug pathways, and the development of co-culture models. They can be used to identify the functional changes and disease mechanisms associated with TDP-43, for drug discovery and the identification of ALS/FTD therapeutic targets, and the development of neuromuscular junction disease models.
Features
Highly pure
iNeu Motor Neurons ALS TDP43 are highly pure cholinergic Human motor neurons that are ≥90% β-III tubulin+/Nestin- and ≥70% Isl1/2 at 14 days in vitro. These proteins also express characteristic markers of motor neurons including Isl1, AchR, C9or72, VAChT, and FoxP1.

Homogenous and reproducible
iNeu Motor Neurons ALS TDP43 undergo rigorous quality control testing ensure the same performance with every batch of cells.

Rapid results
iNeu Motor Neurons ALS TDP43 are shipped cryopreserved with optimized media. Simply thaw and use.
Cell Types
Motor Neuron
Applications
Cell Assay
Species
Human
Size
1 Vial: ≥ 3 x 10^6 cells/vial
Tissue Source
Blood
Age
50-59
Mycoplasma Testing
The cell line has been screened using the Mycoplasma Detection Kit to confirm the absence of Mycoplasma contamination.
Shipping
Dry ice.
Storage
Liquid Nitrogen
Research Use Only
For research use only
Quality Control
All cells test negative for mycoplasma, bacteria, yeast, and fungi.
Target
5-HT1A
Official Name
HTR1A
Alternative Names
5-HT1A receptor; ADRB2RL1; ADRBRL1; 5-HT1A; 5-hydroxytryptamine (serotonin) receptor 1A; 5HT1A; RAT5HT1A; 5-HT-1A; 5-hydroxytryptamine receptor 1A; serotonin receptor 1A; 5-HT1A receptor; Gpcr18
Gender
Male
Donor Health
ALS/FTD
Ethnicity
Caucasian

For Research Use Only. Not For Clinical Use.
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