TARDBP and proteostasis deficiencies: In the frontal cortex of the TDP-43-M323K mouse model of TDP-43 proteinopathy, our lipidomic analysis revealed that the cholesterol metabolism was clearly altered in the mutant brains, pointing towards an accumulation of cholesterol esters and triglycerides in the form of lipid droplets, as well as a reduction in the levels of free cholesterol.