Table 4.

Treatment studies using AICAR for the activation of mitochondrial biogenesis in mouse models

Mouse model	Effect on phenotype	Evidence for mitochondrial biogenesis	Reference
Surf1−/− model of COX deficiency	Improved biochemical phenotype, no motor impairment to improve	+	Viscomi et al. (2011)
ACTA-Cox15−/− model of mitochondrial myopathy	Improved biochemical phenotype without improved motor impairment	+	Viscomi et al. (2011)
Sco2KO/KI model of COX deficiency	Improved biochemical phenotype and improved motor impairment	+	Viscomi et al. (2011)
mdx model of DMD (skeletal muscle)	Amelioration of muscle pathology. May have positive effect on muscle function	+	Ljubicic et al. (2011); Jahnke et al. (2012)
mdx model of DMD (diaphragm)	Amelioration of muscle pathology. Evidence of increased mitophagy	−	Pauly et al. (2012)