ALS-associated mutations and the antiviral response
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that is associated with genetic mutations, most commonly C9orf72. ALS shares many similarities with acute flaccid myelitis (AFM), which is caused by enteroviruses. We hypothesize that C9-ALS associated mutations will alter the antiviral response. Our methods involved two cell lines, C9-ALS mutated cells and control fibroblasts. We infected both cell lines with poliovirus and harvested RNA at 4, 8, 24, and 48hpi. Poliovirus gene expression was analyzed using RT qPCR data. The preliminary data found higher gene expression in C9-ALS mutated cells. Our experiments showed mixed gene expression between the two cell lines. The exception is 8hpi, which may have higher poliovirus gene expression in the C9-ALS cell line. More experiments are needed to conclude that there is an altered antiviral response in the C9-ALS associated cell line. Future experiments will need to be done using iPSC’s motor neurons for a more physiologically relevant environment.