Reflecting also the essential role of Opa1 in cellular energy homeostasis, previous studies in mouse models of skeletal muscle atrophy [51], prion disease [52], and liver dysfunction (Li L. et al., 2019; Wu W. et al., 2019; Lee et al., 2020) showed that Opa1 protects myocytes, neurons, and hepatocytes by improving mitophagic flux and mitochondrial dynamics and metabolism. Here, OPA1 is linked to prion disease.