So far, metabolomics analysis has proven effective in identifying particular metabolic pathways affected in the disease progression, pathways which could then be targeted and compensated through therapeutic manipulation.176 Although metabolomic screening has been mainly focused on patients bearing mutations in SOD1 to the detriment of other genetic causes of ALS, in vitro and in vivo experiments shed some light on the association between TDP-43 pathology and metabolic alterations. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.