Peroxisome proliferator-activated receptor γ coactivator 1α (PGC-1α) acts as a master regulator of mitochondrial biogenesis by regulating mitochondrial content (21); therefore, we assessed changes in PGC-1α expression in kidney tissue from ADPKD model animals to determine whether a lower renal mtDNA copy number was associated with decreased PGC-1α expression. Here, PPARGC1A is linked to autosomal dominant polycystic kidney disease.