The Effects of SOD-mutations Associated With Familial ALS on Motor Neuron-like Cell Line, NSC-34.

Undergraduates: Moza Hamud, Matt Beck

Faculty Advisor: Mohanish Deshmukh
Department: Biology

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease where motor neurons die and eventually leads to the inability of the brain to initiate and control muscle movement. Approximately 10% of ALS cases are classified as familial ALS, as opposed to sporadic ALS (¿¿¿90%). Familial ALS has been associated with certain genetic mutations, many of which affecting the enzyme Superoxide Dismutase (SOD) and possibly leading to its destabilization. Therefore, drugs that stabilize SOD offer a potential therapeutic window for ALS. This study examines the effect of introducing several SOD-mutation plasmids into the motor neuron-like cell line, NSC-34, and assessing cell death. Our goal is to use this assay to test several SOD-stabilizing drugs on their ability to reduce cell death in the presence of SOD-destabilizing mutations.