TP53 and Li-Fraumeni syndrome: In comparison, oncogenic p53 mutations found in the Li-Fraumeni syndrome increase in vivo skeletal muscle oxidative phosphorylation in humans (Wang et al., 2013), while mitochondrial respiration and content of electron transport chain proteins is increased in primary myoblasts from Li-Fraumeni carriers and in mice carrying a p53 R712H polymorphism (Wang et al., 2013).