TARDBP and proteostasis deficiencies: Reduced mitochondrial ATP synthesis due to suppressed complex I activities, reduced mitochondrial membrane potential, and increased ROS, was proposed to result in the observed severe structural deformations of the mitochondria in patients with TDP-43 proteinopathy, as well as in HEK293 cells with induced exogenous TDP-43 expression (wild-type and A315T variant) and in transgenic flies (wild-type and A315T variant) [37].