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In the mitochondrial matrix, acyl-CoA undergoes -oxidation to produce acetyl-CoA, which then enters the citric acid cycle and is fully oxidized to generate large amounts of ATP (75)

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The fatty acids separating (triglycerides derivative) modified by adipose tissues among the metabolism of plasma cholesterol, several tissues and successive intracellular procedures related and dependent on accessibility of lipid [605]

After treatment with carglumic acid, there were no clinical symptoms in 8 of 30 (27%) episodes with available baseline and endpoint data