In recent years, accumulated evidence suggests a key role of mitochondria-related transcriptional regulators peroxisome proliferator-activated receptor gamma coactivator 1-alpha and transcription factor EB (PGC-1α, TFEB, respectively) and of carnitine palmitoyl-transferase 1A (CPT1A) in kidney disease (Figure 2). Here, PPARGC1A is linked to kidney disorder.