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Conditions for which this effect has been demonstrated are: glutaric aciduria II, methyl malonic aciduria, propionic acidemia, and medium chain fatty acylCoA dehydrogenase deficiency

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Calculations of key parameters of the mitochondrial function were performed, consisting of basal respiration (BR, OCR initial OCR rot/AA ), ATP production (ATP, OCR initial - OCR oligo ), proton leak (PL, OCR oligo OCR rot/AA ), maximal respiration (MR, OCR FCCP OCR rot/AA ), spare respiratory capacity (SRC, OCR FCCP - OCR initial ), spare respiratory capacity [%] (SRCP, MR/BR*100), non-mitochondrial respiration rate (NMRR, OCR rot/AA ), and coupling efficiency [%] (CE, ATP/BR*100)

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