Mice overexpressing PHB1 developed obesity associated with increased mitochondrial biogenesis (Ande et al., 2014) in the presence of high copy numbers of mtDNA and mitochondrial proteins of transcription factors regulating mitochondrial biogenesis and nuclear genes encoding protein structures of the OXPHOS complex (PGC-1α, NRF2, OPA1, DRP1, TFAM) (Ande et al., 2014). This evidence concerns the gene PHB1 and obesity due to melanocortin 4 receptor deficiency.