Research Information System
Medeniyet Medical Journal, vol.34, no.2, pp.123-129, 2019 (Scopus)
Objective: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder affecting spinal and cortical motor neurons. Motor neuron degeneration leads to progressive muscular atrophy. Als2 (alsin) is one of the causative genes of ALS and UXT gene is an interacting partner of Als2. Since UXT is a known cofactor in NF-κB enhancesome, we aimed here to compare the effects of Als2 silencing on NF-κB activity by using N2a and C2C12 cell lines. Methods: Mouse neuroblastoma cells (N2a) and myoblast cell lines (C2C12) were used. Als2 was silenced by RNAi and UXT, A20 and IL8 expressions were measured by qRT-PCR. Data were statistically evaluated by Student t-test. Results: A 73.4% decrease was observed in Als2 expression in N2a cells, whereas 73.7% decrease in the C2C12 cells was found. UXT expression in Als2-silenced N2a cells was found to be decreased by 40%, while a 9-fold increase in C2C12 cells was detected. IL8 expression demonstrated a 84% decrease in N2a cells. On the other hand,IL8 expression increased 50-fold in C2C12 cells. A20 expression increased by 65% in N2a cell line, and increased 10 fold in C2C12 cell line. Conclusion: We have concluded that silencing Als2 gene over UXT on exerts different effects on NF-κB pathway We arrived at this conclusion because changes in the expressions of the IL8 and A20 genes differ in both cell lines. In addition, the link between Als2 and UXT is shown again in both cell lines and it is understood that the silencing Als2 may give rise to different results as for neuronal cells and muscle cells.