We propose that the similarities in OxPhos expression between genotypes may be due, in part, to the enhanced expression of key transcriptional regulators of mitochondrial genes at the nDNA in SMA skeletal muscle, such as Nrf2 and Sirt1, as well as at the mtDNA, including Tfam and p53. The gene discussed is TP53; the disease is proximal spinal muscular atrophy.