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ALS Model - FUS-TLS Stress Granules Assay

Data Sheet

Amyotrophic lateral sclerosis (ALS) is one of the most common degenerative disease of the motor neuron system. FUS/TLS (fused in sarcoma/translocated in liposarcoma) and TDP-43 are integrally involved in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia.
Stress granules (SGs) are granules of RNA and proteins formed in the cytosol of the cell under stressful conditions. Disease-linked mutations of FUS increase its propensity to aggregate and to form SGs by preventing nuclear translocation.

In normal conditions, FUS/TLS protein is predominantly localized in the nucleus. In the presence of an oxidative insult like Sodium arsenite, FUS/TLS increases its cytoplasmic localization and it is accumulated in Stress granules.

Assay Details

Cells will be induced to express the FUS/TLS-tGFP. After that, cells will be incubated with the compounds to be tested during 24 hours using Arimoclomol or Riluzole at 10 uM as positive control. Then, cells will be treated with 300 µM sodium arsenite. The FUS/TLS-tGFP stress granules will be quantified using the BD Pathway HCS Reader and Attovision Compartimentalization Software.



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