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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-43 A315T mice, a mouse model which recapitulates many aspects of TDP-43 pathology observed in ALS and ALS/FTLD patients 19

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