CDK5RAP1 and maternally-inherited diabetes and deafness: Since the mt-tRNALeu(UUG) mutation causes mitochondrial dysfunction and oxidative stress [143], the authors proposed that enhanced ROS levels may result in 4Fe-4S oxidation and CDK5RAP1 inactivation, pointing to oxidative stress-mediated CDK5RAP1 inactivation as an additional contributor to mitochondrial failure in MIDD and mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes [97] (Figure 2).