Consistently, we found extensive overlap between the DELE1 mt-ISR signature in the skeletal muscle of the IMMD mouse model of myopathy and genes upregulated in three cohorts of patients with MM, which included patients with mutations in Twinkle, TK2, and mtDNA (Hathazi et al, 2020; Pirinen et al, 2020; Kalko et al, 2014). Here, TK2 is linked to myopathy.