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Schematic overview of the proteins and pathways involved in mtDNA maintenance. Zooming in on the mtDNA allows the identification of factors involved in mtDNA replication and repair (A) including: POLG, Twinkle, DNA2, MGME1; on the right (B) are the proteins assumed to affect the import and metabolism of the mitochondrial dNTP precursors; on the left (C) are the supposed localization of key factors ruling mitochondrial dynamics (MFN2, OPA1).
