COX5A and hyperinsulinemic hypoglycemia, familial, 4: To test this, Viscomi and colleagues performed an elegant study where three different recombinant mouse models (Surf1 KO, Sco2 KO/Knock-in (IN), muscle-specific Cox15 KO) with COX deficiency in skeletal muscle, therefore presenting mitochondrial myopathy [118] were tested with 5-aminoimidazole-4-carboxamide ribonucleoside (AICAR), which is an AMPK agonist [119].