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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

This isozyme, located on the inner face of the inner mitochondrial membrane, regenerates lipoyl-CoA and releases free carnitine into the matrix

Tubular epithelial cell necrosis and apoptosis (nearly 25% of the examined tissue) were observed (Fig

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They performed wild-type or C/EBP LIP homozygous bone marrow transplants into lethally irradiated mice

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