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Proteomic profiling by TMT based LC-MS 3 analysis On the background of lipid accumulation, mitochondrial proliferation, abnormal mitochondrial structure, apparently reduced activity of Complex II and IV as seen on enzyme histochemistry (COX and SDH), and accumulation of acylcarnitines of various lengths in blood, all pointing toward a mitochondrial dysfunction, we studied the expression of mitochondrial proteins in the proteome of the muscle-biopsy specimens