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These changes not only resulted in a substantial decrease in mitochondrial activity, but were also coupled with a reduction in the expression of MFN2 and OPA1, exacerbating impaired mitochondrial fusion and increasing mitochondrial fragmentation ( 5.3 Mitochondrial biogenesis and COPD Mitochondrial biogenesis is crucial for maintaining cellular homeostasis and it enables cells to replace damaged or aged mitochondria with new ones, thus maintaining normal morphology, structure, and function of mitochondria

Treatment Options for Carnitine Deficiency When someone has primary carnitine deficiency, they usually need to take high doses of oral L-carnitine (100 to 200 mg/kg) for the rest of their life