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The accumulation of LCACs during mitochondrial FAO disorders can inhibit the activity of PPAR/PGC1, destroy the regeneration of peroxisomes, lead to increases in BCAA and ROS levels, and activate the nuclear factor erythroid 2-related factor 2 (Nrf2) pathway
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H 2 O 2 content determination The estimation of H 2 O 2 was determined suggested by Okuda et al
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