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Acknowledgements We thank Elisabeth Rushing (Institute of Neuropathology, University Hospital Zurich) for support with the analysis of histological sections and Friedhelm Bladt and Frank Jhrling (Merck, Darmstadt, Germany) for providing EMD1214063 and for helpful discussions

The ROS-sensitive microRNA-9/9* controls the expression of mitochondrial tRNA-modifying enzymes and is involved in the molecular mechanism of MELAS syndrome