Importantly, Cu deposition in the liver and brain can cause cellular damage and severe hepatic and neurological symptoms.67 Disrupted mitochondrial membranes and increased oxidative damage have been observed in the Long–Evans Cinnamon rat, an animal model of Wilson disease.264 In addition, Tsvetkov et al. have shown that Atp7b knockout mice exhibit reduced levels of lipoylated enzymes and Fe–S cluster proteins compared to wild-type mice,2 reminiscent of the cellular phenotype induced by Cu ionophores (Fig. 3). This evidence concerns the gene ATP7B and Wilson disease.