Description
ALS Model: Red Fluorescent SOD1-A4V HEK293 Cell Line
Mutations in superoxide dismutase 1 gene (SOD1) can cause amyotrophic lateral sclerosis (ALS), a neurodegenerative disease. The detection of SOD1-positive inclusions in familial ALS patients suggests the role of SOD1 aggregation underlying the pathology of familial ALS. All SOD1 mutant proteins exhibit a higher aggregation propensity than wild-type SOD1.
We developed HEK293 cell line stably expressing red fluorescent A4V mutant of SOD1 for an aggregation assay. In the assay, we incubate cells with test compounds for 24 hours in order to evaluate their effect in preventing SOD1-A4V aggregation. Then, we treat the cells with ALLN, cysteine proteinase inhibitor, to induce aggregation. After overnight incubation, the cells are fixed and the red fluorescent SOD1-A4V aggregates are quantified by image analysis.
Also available:
- HEK293 Cell line stably expressing Green fluorescent SOD1 (wild type)
- HEK293 Cell line stably expressing Red fluorescent SOD1 (wild type)
