Figure 10.

Rescue of mutant TDP-43 induced mitochondrial dysfunction by Mfn2 overexpression. Motor neurons were seeded on 24-well plates and transfected with GFP tagged Mfn2 or BFP tagged mutant TDP-43 or co-transfected with GFP tagged Mfn2 and BFP tagged mutant TDP-43 at DIV7. At DIV9, the mitochondrial ROS and mitochondrial membrane potential (MMP) were measured by mitoSOX and TMRM respectively. (A and B) Quantification of the relative fluorescent intensity of mitoSOX (A) and TMRM (B) in cell bodies of motor neurons. At least 50 neurons were analyzed in each experiment and experiments were repeated three times. Data are means ± s.e.m. Statistics: one-way analysis of variance (ANOVA) followed by Tukey's multiple comparison test. *P < 0.05, compared with control neuron and ^#P < 0.05, compared with neurons expressing TDP-43 M337V with normal TDP-43 localization.