Studies regarding Tar DNA-binding protein 43 (TDP-43), which accumulates in certain neurodegenerative diseases such as ALS and Frontotemporal dementia (FTD), showed that it was found in mitochondria where it led to aberrant morphology and mitophagy mediated by Parkin, increased production of ROS, mitochondrial membrane permeabilization and impaired oxidative phosphorylation [195,259]. Here, PRKN is linked to frontotemporal dementia.