Superoxide Dismutase 1 and Cell Death in Familial ALS
Familial amyotrophic lateral sclerosis (ALS) is a progressive, genetic neurodegenerative disease affecting motor neurons in the brain and spinal cord. Mutations of the gene encoding superoxide dismutase 1 (SOD1) have been implicated in ~20% of cases. SOD1 eliminates reactive oxygen atoms that donate electrons to other proteins, damaging cells. While research into fALS has primarily focused on motoneuron dysfunction directly, recent research has shown that blood brain barrier (BBB) glial dysfunction may cause motoneuron death. The BBB is composed of blood vessel endothelial cells and surrounding glia, which prevents entry of unwanted molecules from the blood into the CNS. We hypothesize that alterations in SOD1 function in glial cells of the Drosophila BBB alters its integrity and contributes to animal death in this model of ALS. Our results suggest that fly lifespan is differentially affected by SOD1 knockdown in glia vs neurons, with a knockdown in glia being more detrimental.
Keywords: Drosophila, SOD1, cell death, ALS, blood brain barrier, glia
Topic(s):Biology
Presentation Type: Oral Presentation
Session: 405-4
Location: MG 2001
Time: 2:45