Further to this, TDP-43 CTFs are typically not detected in the ALS spinal cord (Neumann et al., 2006; Igaz et al., 2008; Uchida et al., 2012; Kwong et al., 2014), despite the dramatic tissue loss associated with disease progression (Branco et al., 2014). Here, TARDBP is linked to amyotrophic lateral sclerosis.