From a biological point of view, the significance of this finding could be very high because, although rescue of TDP-43 pathology did not occur in a yeast model of disease following ablation of the homologous yeast gene (Aif1p) (56), elevated levels of this factor have been observed in specific molecular subtypes of ALS patients (57), in the cortex of mice subjected to mild traumatic brain injury (58), and very recently in Atxn-CAG100 knock-in mice that eventually develop TDP-43 pathology (59). Here, TARDBP is linked to amyotrophic lateral sclerosis.