Regulation of mitochondrial FoF1ATPase activity by Sirt3-catalyzed deacetylation and its deficiency in human cells harboring 4977Â bp deletion of mitochondrial DNA

► We demonstrated a role of Sirt3 in the regulation of acetylation and activity of mitochondrial FoF1ATPase. ► The OSCP subunit of FoF1ATPase was identified as a new target of Sirt3. ► ROS overproduction led to a decline in the expression level of Sirt3 and the impairment of FoF1ATPase. ► Oxidative stress is involved in the Sirt3 deficiency in human cells harboring the 4977 bp deletion of mtDNA.