Moreover, in two different cellular models (SH-SY5Y and human fibroblast) L-BMAA treatment determines a significant TDP-43 mislocalization into the cytoplasm (Figures 4, 5) and a significant accumulation of insoluble aggregates (Figure 4), two typical pathological signs of TDP-43 proteinopathy. The gene discussed is TARDBP; the disease is proteostasis deficiencies.