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Research has revealed a unique protease activation phenomenon in MELAS: LONP1 exhibits subcellular compartmentalized aggregation in primary cells from patients with MELAS, with its protein abundance particularly elevated in cell populations enriched with mutant mtDNA

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Nrf2, the innate transcription factor, regulates synthesis and activity of several antioxidant enzymes to help resolve OxS, and prevent tissue damage in lungs 678