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Most of these therapeutic approaches require further investigation in large-scale, blinded, placebo-controlled randomised studies to achieve adequate evidence-based and clinically reliable recommendation grades [476-481]

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Other studies reported lactic acidosis and increased alanine levels, it is highly recognized that high concentrations of lactate can inhibit mitochondrial respiration and disbalance the redox equilibrium (Oliveira et al., 2005)

In addition, investigators are exploring therapeutic strategies to address this metabolic feature of the disease

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Plasma levels can be raised using L-carnitine maintenance therapy, and the dose is adjusted based on both the response and the plasma levels