Redox Signaling in Sickle Cell Disease. Curr Opin Physiol 2019 Jun;9:26-33
Date
06/27/2019Pubmed ID
31240269Pubmed Central ID
PMC6592428DOI
10.1016/j.cophys.2019.04.022Scopus ID
2-s2.0-85065766645 (requires institutional sign-in at Scopus site) 14 CitationsAbstract
Sickle cell disease (SCD) is characterized by chronic hemolysis and repeated episodes of vascular occlusion leading to progressive organ injury. SCD is characterized by unbalanced, simultaneous pro-oxidant and anti-oxidant processes at the molecular, cellular and tissue levels, with the majority of reactions tipped in favor of pro-oxidant pathways. In this brief review we discuss new findings regarding how oxidized hemin, hemolysis, mitochondrial dysfunction and the innate immune system generate oxidative stress while hemopexin, haptoglobin, heme oxygenase-1 (HO-1) and nuclear factor erythroid 2-related factor 2 (Nrf2) may provide protection in human and murine SCD. We will also describe recent clinical trials showing beneficial effects of antioxidant therapy in SCD.
