Considering that ALS is generally thought to progress as a consequence of genetic susceptibility and environmental influences, and that TDP-43 has been observed as the major component of ubiquitinated inclusions in post-mortem tissues of ALS patients and patients with frontotemporal dementia (41), we decided to extend our analysis by evaluating the effects of L-BMAA or LSC-MaCe exposure in different ALS cellular models (49). This evidence concerns the gene TARDBP and frontotemporal dementia.