FUS and amyotrophic lateral sclerosis: Moreover, these abnormalities are not restricted to the mutant SOD1 pathogenesis, also mice overexpressing human TDP-43 have abnormal distribution of mitochondria and changes in mitochondrial dynamics (Xu et al., 2010; Wang et al., 2013), while neuronal cultures expressing ALS-linked FUS mutants contain smaller mitochondria (Tradewell et al., 2012).