Recent in vitro experiments using familial ALS patient-derived induced pluripotent stem (IPS) cells with the M337V TDP-43 mutation showed that astrocytes generated from these IPS cells reveal cytoplasmic inclusions and premature cell death, thereby mimicking histopathological findings of TDP-43 proteinopathies (53). Here, TARDBP is linked to amyotrophic lateral sclerosis.