For example, mitochondrial aggregates were detected in transgenic mice overexpressing WT form of TDP-43 (Xu et al., 2010), numerous mitochondrial defects are present in transgenic mouse models of TDP-43 as well as Betz cells of ALS and ALS/FTLD patients with TDP-43 pathology (Wang et al., 2013; Gautam et al., 2019). Here, TARDBP is linked to amyotrophic lateral sclerosis.