Fluorescent SOD1 Cell Line

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by loss of both; upper and lower motoneurons in the brain and the spinal cord. 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 inclusion formation in motor neurons. Classical neuropathological hallmarks of ALS include ubiquitinated inclusions containing the disordered TDP-43 and FUS proteins; although pathology can be heterogeneous with the appearance of other protein aggregates. Mutations in more than forty genes have been reported to associate with ALS. SOD1 is a ubiquitously expressed protein, existing as a homodimer of 32 kDa. Each monomer is highly structured, and intramolecular disulfide bridges increase their stability. The enzyme catalizes the disproportionation of superoxide species to hydrogen peroxide and dioxygen. Fluorescent SOD1 cell line is a useful tool to assay compounds or analyze their capability to modulate superoxide dismutase 1 activity.