In FRDA, the expression of the encoded protein frataxin was decreased, leading to obstruction of mitochondrial matrix ironsulfur cluster biosynthesis, mitochondrial dysfunction, and mitochondrial iron accumulation, and finally result in increased oxidative stress
While the RNI for maintenance is only 1.5 g daily, therapeutic oral doses typically range from: 50-150 g daily for mild deficiency prevention in at-risk groups 1,000-2,000 g daily for treating confirmed deficiency without malabsorption Some formulations contain up to 5,000 g, though there is no proven additional benefit over 1,000-2,000 g for most patients The reason for this dosage discrepancy lies in absorption efficiency
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What changes over time: the first weeks versus the long haul Weeks 18 (titration): GI side effects peak