Meanwhile, studies on TDP-43 function loss have been conducted on many organisms, including cultured neurons,8 conditional TDP-43 knockout mice,9  Drosophila,10 and zebrafish.11 TDP-43 depletion has been recently reported to promote the cryptic exon of several genes, such as stathmin-2 and UNC13A, which downregulate their transcriptions.12-15 As these molecules are indispensable for neuronal activity, the loss of TDP-43 function has increasingly gained attention as a cause of ALS pathology. Here, TARDBP is linked to amyotrophic lateral sclerosis.