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Sickle Cell Disease: Role of Oxidative Stress and Antioxidant Therapy

1
Biomarkers Unit, Center for Gender-Specific Medicine, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy
2
Department of Clinical and Molecular Medicine, “La Sapienza” University, 00161 Rome, Italy
3
Endocrine-Metabolic Unit, Sant’Andrea University Hospital, 00189 Rome, Italy
4
Core Facilities, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy
*
Author to whom correspondence should be addressed.
Academic Editor: Fabrizio Gentile
Antioxidants 2021, 10(2), 296; https://doi.org/10.3390/antiox10020296
Received: 19 December 2020 / Revised: 8 February 2021 / Accepted: 12 February 2021 / Published: 16 February 2021
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. View Full-Text
Keywords: sickle cell disease; hemoglobin; oxidative stress; antioxidants; red blood cells sickle cell disease; hemoglobin; oxidative stress; antioxidants; red blood cells
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MDPI and ACS Style

Vona, R.; Sposi, N.M.; Mattia, L.; Gambardella, L.; Straface, E.; Pietraforte, D. Sickle Cell Disease: Role of Oxidative Stress and Antioxidant Therapy. Antioxidants 2021, 10, 296. https://doi.org/10.3390/antiox10020296

AMA Style

Vona R, Sposi NM, Mattia L, Gambardella L, Straface E, Pietraforte D. Sickle Cell Disease: Role of Oxidative Stress and Antioxidant Therapy. Antioxidants. 2021; 10(2):296. https://doi.org/10.3390/antiox10020296

Chicago/Turabian Style

Vona, Rosa, Nadia M. Sposi, Lorenza Mattia, Lucrezia Gambardella, Elisabetta Straface, and Donatella Pietraforte. 2021. "Sickle Cell Disease: Role of Oxidative Stress and Antioxidant Therapy" Antioxidants 10, no. 2: 296. https://doi.org/10.3390/antiox10020296

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