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The principle that works: conservative start, slower titration, longer hold periods at each dose

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For instance, in a six-month randomized controlled trial of 72 patients with Friedreichs ataxia (a neurodegenerative disorder characterized by mitochondrial iron accumulation), 20 mg/kg/day DFP was well tolerated but higher doses (40 and 60 mg/kg/day) were associated with increased adverse events and worsening of ataxia symptoms (Pandolfo et al., 2014)

The participation of three families of transporters in mammalian cells involved in the transportation and Antioxidants 2023,12, 834 6 of 24 movement of glutathione has been demonstrated: a family of drug resistance-associated proteins (MRP), cystic brosis transmembrane conductance regulator family (CFTR) and organic-anion-transporting polypeptide family (OATP) (Figure 3) [77,78]