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For Research Use Only. Not For Clinical Use.

STEMOD™ iPSC-derived ALS Disease Model (TARDBP, G298S)(MoCNS-WQ019)

Datasheet

ALS is a severe neurodegenerative condition characterized by loss of motor neurons in the spinal cord and brain. This disease cell model has a M337V mutation in TARDBP gene. it belongs to familial ALS. Generating pluripotent stem cells from fibroblasts, then differentiate into motor neurons . This disease cell model provides a platform to explore disease mechanisms in ALS.

Specifications
Organ System Central Nervous System
Disease Amyotrophic Lateral Sclerosis (ALS)
Target Gene TARDBP
Gene Function TDP-43 encoded by this gene is a transcriptional repressor that binds to chromosomally integrated TAR DNA and TDP-43 is involved in multiple steps of RNA metabolism, including transcription, splicing, and transport of mRNA
Mutation G298S
Phenotype Mutant neurons had elevated levels of soluble and detergent-resistant TDP-43 protein, decreased survival in longitudinal studies, and increased vulnerability to antagonism of the PI5K pathway.
Source Fibroblasts
Cellular Assays Immunocytochemical analysis, Immunoprecipitation
Target Gene
Gene ID 23435
Uniprot ID Q13148

For Research Use Only. Not For Clinical Use.

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