Frontotemporal lobar degeneration (FTLD), a neurodegenerative syndrome in frontal and anterior temporal lobes (Rabinovici and Miller, 2010), and ALS, a motor neuron disorder characterized by degeneration in the upper and lower motor neurons (Prasad et al., 2019), are two distinct diseases that shared a histopathological hallmark: inclusion bodies composed of cytoplasmic deposits of the nuclear TDP-43 protein (Scialo et al., 2021). Here, TARDBP is linked to amyotrophic lateral sclerosis.