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In addition to limiting sulfur through starving cells of cystine, we restricted iron availability by chelating cellular iron with deferoxamine (DFO) 41 or ablated synthesis altogether by silencing expression of the vital Fe-S biosynthetic machinery components, NFS1 and ISCU

It is administered intravenously or intramuscularly by a healthcare professional, with dosing tailored to the individual patients needs and clinical response

Following discovery of the HFE gene [36], population studies demonstrated variable biochemical and clinical manifestations [11, 24, 37, 38]

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The rationale was that hydride transfer to the flavin N5 delivers the proton from the ostensibly non-exchangeable aliphatic 4-position of the NADPH dihydronicotinamide to the amine N5 position of the flavin isoalloxazine that is exchangeable with solvent

10.1172/jci.insight.90777 87 LiQ.WeilandA.ChenX.LanX.HanX.DurhamF.et al (2018a)