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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

The activity was followed by measuring the quantity of radioactive diol formed in the aqueous phase using a scintillation counter (TriCarb 2810 TR, Perkin Elmer, Shelton, CT)

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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