Structural abnormalities of mitochondria have been reproduced in several ALS TDP-43 models; fibroblasts transfected with TDP–43G298S or TDP-43A382T [176], NSC-34 motor neuron like cells transfected with TDP-43Q331K, TDP-43M337V, or with TDP-43-C terminal fragments [61,177,178], HEK293T transfected with TDP-43G298S or TDP-43A382T [176], primary mouse motor neurons transfected with TDP-43Q331K or TDP-43M337V [177], and transgenic TDP-43A315T or TDP-43-C terminal fragment mouse models [179,180,181,182]. Here, TARDBP is linked to amyotrophic lateral sclerosis.