PRKAA1 and obesity due to melanocortin 4 receptor deficiency: Our data identified that AMPK inactivation is a driver of mitochondrial biogenesis and mtDNA heteroplasmy via the LonP1‐ATF5 axis in mature oocytes, providing a therapeutic target to improve oocyte maturation and female fertility, as well as offspring metabolic health, which are impaired due to obesity.