In line with this concept, the pharmacological activation of AMPK using the agonist 5‐aminoimidazole‐4‐carboxamide ribonucleoside (AICAR) has been shown to improve muscle function in different models of mitochondrial myopathy caused by complex IV (CIV) defects [3, 4]. Here, PRKAA1 is linked to Mitochondrial myopathy.