Research Paper Volume 11, Issue 8 pp 2457—2476

Deletion of Tbk1 disrupts autophagy and reproduces behavioral and locomotor symptoms of FTD-ALS in mice

Figure 10. Over-expression of Tbk1 reduces the number and size of mutant SOD1 aggregates. (AB) Western blot analysis of Tbk1, p-Tbk1, SOD1G93A, LC3B, and p62 in NSC-34 cells transfected with mutant SOD1 (mu SOD1; i.e. SOD1G93A) and either Tbk1 or a RFP control; representative blot (A) and summary graph of densitometric quantification data (B) (n = 3); *P < 0.05, compared to control. (CD) Analysis of mutant SOD1 aggregates by confocal microscopy in NSC-34 cells co-transfected with mutant SOD1-GFP (mu SOD1-GFP) and Tbk1 or RFP; representative images (C) and summary graph (D) (n = 10); *P < 0.05, compared to control. (EF) Quantification of soluble and insoluble mutant SOD1 fractions by western blot in cells over-expressing Tbk1 (n = 3); *P < 0.05, compared to control.