Vona Rosa, Sposi Nadia Maria, Mattia Lorenza, Gambardella Lucrezia, Straface Elisabetta, Pietraforte Donatella
Biomarkers Unit, Center for Gender-Specific Medicine, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy.
Department of Clinical and Molecular Medicine, "La Sapienza" University, 00161 Rome, Italy.
Antioxidants (Basel). 2021 Feb 16;10(2):296. doi: 10.3390/antiox10020296.
Sickle cell disease (SCD) is the most common hereditary disorder of hemoglobin (Hb), which affects approximately a million people worldwide. It is characterized by a single nucleotide substitution in the β-globin gene, leading to the production of abnormal sickle hemoglobin (HbS) with multi-system consequences. HbS polymerization is the primary event in SCD. Repeated polymerization and depolymerization of Hb causes oxidative stress that plays a key role in the pathophysiology of hemolysis, vessel occlusion and the following organ damage in sickle cell patients. For this reason, reactive oxidizing species and the (end)-products of their oxidative reactions have been proposed as markers of both tissue pro-oxidant status and disease severity. Although more studies are needed to clarify their role, antioxidant agents have been shown to be effective in reducing pathological consequences of the disease by preventing oxidative damage in SCD, i.e., by decreasing the oxidant formation or repairing the induced damage. An improved understanding of oxidative stress will lead to targeted antioxidant therapies that should prevent or delay the development of organ complications in this patient population.
镰状细胞病（SCD）是最常见的血红蛋白（Hb）遗传性疾病，全球约有100万人受其影响。其特征是β-珠蛋白基因中的单核苷酸取代，导致产生异常的镰状血红蛋白（HbS），并产生多系统后果。HbS聚合是SCD中的主要事件。Hb的反复聚合和解聚导致氧化应激，这在镰状细胞病患者的溶血、血管闭塞及随后的器官损伤的病理生理学中起关键作用。因此，活性氧化物质及其氧化反应的（终）产物已被提议作为组织促氧化状态和疾病严重程度的标志物。尽管需要更多研究来阐明它们的作用，但抗氧化剂已被证明可通过预防SCD中的氧化损伤，即通过减少氧化剂形成或修复诱导的损伤，有效减轻该疾病的病理后果。对氧化应激的进一步了解将带来有针对性的抗氧化疗法，有望预防或延缓该患者群体器官并发症的发生。
