Description
This ALS model consists in a U2OS cell line stably expressing green fluorescent TDP-43 and red fluorescent FUS/TLS. This novel fluorescence cell-based assay cell line allows the quantification of both TDP-43 and FUS/TLS stress granules in high content screening.
- We offer 2 vials for each order and each vial contains 3 million cells;
- These cells proliferate in vitro;
- Guaranteed to further expand for more than 30 passages following the instructions provided.
Amyotrophic lateral sclerosis (ALS):
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder. ALS and other neurodegenerative disorders, such as Alzheimer’s and Parkinson’s disease; are characterized by defects in protein processing resulting in protein misfolding, mislocalization and also inclusion formation in motor neurons. Classical neuropathological hallmarks of ALS include ubiquitinated inclusions containing the disordered TDP-43 and FUS proteins.
TDP-43:
TDP-43 is a highly-conserved, ubiquitously-expressed ribonucleoprotein with multiple roles in nucleic acid metabolism. TDP-43 mainly resides in the nucleus. However, under pathological conditions, TDP-43 mislocalizes to the cytosol. TDP-43 has been identified as one of the primary component of ubiquitinated and hyper-phosphorylated cytosolic aggregates observed in ALS patients.
TDP-43 is related with both familial and sporadic forms of ALS. Its aggregation is considered a hallmark of ALS. It is observed in approximately 97% of all ALS patients regardless of the mechanisms of disease onset.
Fused in sarcoma (FUS):
Fused in sarcoma (FUS) is a multifunctional RNA-binding protein. It plays a role in many RNA metabolism pathways. FUS is genetically and pathologically linked to uncommon but very aggressive forms of ALS and frontotemporal dementia (FTD). As TDP-43, FUS is mainly localized in the nucleus and under pathological conditions it is aggregated in the cytosol.
To date, more than 50 different FUS mutations have been identified in patients with ALS. This accounts for approximately 4% of familial cases and fewer than 2% of patients with sporadic ALS.
