Kumfu Sirinart, Chattipakorn Siriporn C, Chattipakorn Nipon
Cardiac Electrophysiology Research and Training Center, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand.
Cardiac Electrophysiology Unit, Department of Physiology, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand.
Exp Biol Med (Maywood). 2022 Apr;247(7):574-583. doi: 10.1177/15353702221076397. Epub 2022 Feb 7.
Iron overload can be the result of either dysregulated iron metabolism in the case of hereditary hemochromatosis or repeated blood transfusions in the case of secondary hemochromatosis (e.g. in β-thalassemia and sickle cell anemia patients). Under iron overload conditions, transferrin (Tf) saturation leads to an increase in non-Tf bound iron which can result in the generation of reactive oxygen species (ROS). These excess ROS can damage cellular components, resulting in the dysfunction of vital organs including iron overload cardiomyopathy (IOC). Multiple studies have demonstrated that L-type and T-type calcium channels are the main routes for iron uptake in the heart, and that calcium channel blockers, given either individually or in combination with standard iron chelators, confer cardioprotective effects under iron overload conditions. Treatment with antioxidants may also provide therapeutic benefits. Interestingly, recent studies have suggested that mitochondrial dynamics and regulated cell death (RCD) pathways are potential targets for pharmacological interventions against iron-induced cardiomyocyte injury. In this review, the potential therapeutic roles of iron chelators, antioxidants, iron uptake/metabolism modulators, mitochondrial dynamics modulators, and inhibitors of RCD pathways in IOC are summarized and discussed.
铁过载可能是遗传性血色素沉着症中铁代谢失调的结果,也可能是继发性血色素沉着症(如β地中海贫血和镰状细胞贫血患者)中反复输血的结果。在铁过载条件下,转铁蛋白(Tf)饱和度增加会导致非转铁蛋白结合铁增加,进而可能产生活性氧(ROS)。这些过量的ROS会损害细胞成分,导致包括铁过载心肌病(IOC)在内的重要器官功能障碍。多项研究表明,L型和T型钙通道是心脏摄取铁的主要途径,并且钙通道阻滞剂单独使用或与标准铁螯合剂联合使用时,在铁过载条件下具有心脏保护作用。抗氧化剂治疗也可能带来治疗益处。有趣的是,最近的研究表明,线粒体动力学和调节性细胞死亡(RCD)途径是针对铁诱导的心肌细胞损伤进行药物干预的潜在靶点。在这篇综述中,总结并讨论了铁螯合剂、抗氧化剂、铁摄取/代谢调节剂、线粒体动力学调节剂以及RCD途径抑制剂在IOC中的潜在治疗作用。