Whereas there has been some debate about whether TDP-43 pathology occurs in SOD1-associated ALS (Mackenzie et al, 2007; Tan et al, 2007), recent studies have identified cytoplasmic TDP-43 inclusions in motor neurons from SOD1-associated ALS patients (Okamoto et al, 2011; Jeon et al, 2019), and an increase in triton-insoluble fraction (TIF) of TDP-43 in the spinal cord at the end stage of the disease in SOD1G93A mice (Shan et al, 2009; Sumi et al, 2009; Marino et al, 2015; Jeon et al, 2019). Here, TARDBP is linked to amyotrophic lateral sclerosis.