TARDBP and amyotrophic lateral sclerosis: Several animal models in which wild-type TDP-43 is overexpressed result in neurodegeneration similar to that observed in ALS, accumulate mitochondria in cytoplasmic inclusions in MNs, lack mitochondria in motor axon terminals or exhibit abnormal juxtanuclear aggregates of mitochondria in spinal MNs, suggesting that wild-type TDP-43 impairs mitochondrial function [31,106,107].