Table 2.

Mouse models studies trialling PPAR pan-agonist bezafibrate for the activation of PGC-1α

Mouse model	Effect on phenotype	Evidence for mitochondrial biogenesis	Reference
Cox10 model of mitochondrial myopathy	Prolonged lifespan and delayed onset of myopathy	+	Wenz et al. (2008)
Surf1−/− model of COX deficiency	Loss of body weight, hepatomegaly	−	Viscomi et al. (2011)
ACTA-Cox15−/− model of mitochondrial myopathy	Lethality within 48 h	−	Viscomi et al. (2011)
Peo1 Deletor mouse model of late-onset mitochondrial myopathy	Loss of body weight, hepatomegaly, reduction in body temperature, reduction in COX-negative fibres and mtDNA deletion load	−	Yatsuga and Suomalainen (2012)
Polg Mut mouse model of mitochondrial disease/premature ageing	Delay in hair loss, reduction in skin damage, reduction in spleen size, reduction of body weight, hepatomegaly	−	Dillon et al. (2012a)
Forebrain-specific ΔCox10 mice, a model of mitochondrial encephalopathy	Overall amelioration of the phenotype including attenuation of the loss of motor function, protection of brain damage, reduction of loss of bodyweight	+	Noe et al. (2013)
R6/2 model of HD	Increase in motor performance, increase in lifespan. Amelioration of phenotype in brain, skeletal muscle and BAT. Slight increase in liver weight and lipid vacuolization	+	Johri et al. (2012)
P301S transgenic mouse model of AD	Attenuation of locomotor and anxiety abnormalities, histological improvement in the brain pathology, improvement in lipid vacuoles in BAT, reduced body weight	+ (BAT)	Dumont et al. (2012)