Hallmark neuropathologic features in ALS are the loss of upper and lower motor neurons (UMN/LMNs) and their fibers in the primary motor cortex, brainstem and anterior horns of the spinal cord (Saberi et al., 2015 ▸), together with the presence of phospho­rylated TAR DNA-binding protein 43 (TDP-43), positive cytoplasmic inclusions in both MNs and glial cells (Brettschneider et al., 2013 ▸). Here, TARDBP is linked to amyotrophic lateral sclerosis.