The primary objective of this study was to elucidate whether the myelin alterations observed in patients with neurodegenerative diseases associated with TDP-43 proteinopathy, as well as in mouse models carrying TDP-43 mutations, such as our TDP-43-M323K model, were at least in part a result of TDP-43 dysfunction that affected lipid metabolism. This evidence concerns the gene TARDBP and neurodegenerative disease.