However, administration of GW4869 in a mouse model of ALS expressing human mutant TDP-43A315T (autosomal mutation of ALS), appeared to worsen disease phenotypes, increase levels of insoluble TDP-43, and cause cytoplasmic accumulation of TDP-43 in neurons (Iguchi et al., 2016). Here, TARDBP is linked to amyotrophic lateral sclerosis.