Bou-Fakhredin Rayan, De Franceschi Lucia, Motta Irene, Eid Assaad A, Taher Ali T, Cappellini Maria Domenica
Department of Clinical Sciences and Community Health, University of Milan, 20122 Milan, Italy.
Department of Medicine, University of Verona, and Azienda Ospedaliera Universitaria Verona, 37128 Verona, Italy.
Antioxidants (Basel). 2022 May 13;11(5):967. doi: 10.3390/antiox11050967.
β-thalassemia and sickle cell disease (SCD) are inherited hemoglobinopathies that result in both quantitative and qualitative variations in the β-globin chain. These in turn lead to instability in the generated hemoglobin (Hb) or to a globin chain imbalance that affects the oxidative environment both intracellularly and extracellularly. While oxidative stress is not among the primary etiologies of β-thalassemia and SCD, it plays a significant role in the pathogenesis of these diseases. Different mechanisms exist behind the development of oxidative stress; the result of which is cytotoxicity, causing the oxidation of cellular components that can eventually lead to cell death and organ damage. In this review, we summarize the mechanisms of oxidative stress development in β-thalassemia and SCD and describe the current and potential antioxidant therapeutic strategies. Finally, we discuss the role of targeted therapy in achieving an optimal redox balance.
β地中海贫血和镰状细胞病（SCD）是遗传性血红蛋白病，会导致β珠蛋白链出现数量和质量上的变化。这些变化进而导致生成的血红蛋白（Hb）不稳定，或导致珠蛋白链失衡，从而在细胞内和细胞外影响氧化环境。虽然氧化应激并非β地中海贫血和SCD的主要病因，但它在这些疾病的发病机制中起着重要作用。氧化应激的发生背后存在不同机制；其结果是细胞毒性，导致细胞成分氧化，最终可导致细胞死亡和器官损伤。在本综述中，我们总结了β地中海贫血和SCD中氧化应激发生的机制，并描述了当前和潜在的抗氧化治疗策略。最后，我们讨论了靶向治疗在实现最佳氧化还原平衡中的作用。
