Since mitochondrial defects have been broadly observed in many different diseases in which TDP-43 pathology is commonly detected, and because mitochondria are crucially important for maintaining metabolomic homeostasis, one of the key questions that remains unanswered is: “What are the metabolomic changes that occur with respect to TDP-43 pathology?” We investigated the metabolomic perturbations that occur in the motor cortex of prp-TDP-43A315T mice, a mouse model which recapitulates many aspects of TDP-43 pathology observed in ALS and ALS/FTLD patients19. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.