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Acknowledgements We thank all the researchers who have spent their time and effort in trying to understand the complexity of this issue, and we extend our apologies if we could not accommodate a particular work in this review because of space constraints

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Low v-ATPase-inhibition increases m and enhances cell fitness under mitochondrial mistranslation in multiple cell-types and mitochondrial disease patient-derived fibroblasts To confirm that the suppressed cell growth defect under ACT-induced mistranslation was due to v-ATPase deficiency, we used the highly selective v-ATPase inhibitors concA and bafA on WT cells