Similarly, the addition of cerebrospinal fluid from ALS/FTD patients (Ding et al., 2015), detergent-insoluble fractions of ALS-disease brains (Nonaka et al., 2013) or insoluble phosphorylated TDP-43 from post-mortem brain and spinal cord tissue (Smethurst et al., 2016), results in misfolding of TDP-43 when added to human cell lines. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.