We detected similar alterations of metabolic pathways in a clinical trial using bezafibrate in skeletal muscle of patients with m.3243A>G‐related mitochondrial myopathy (Steele et al, 2020), where the number of complex IV deficient muscle fibres decreased and cardiac function improved, while FGF21 and GDF15 increased in all patients. Here, GDF15 is linked to Mitochondrial myopathy.