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Redox Signaling in Sickle Cell Disease. Curr Opin Physiol 2019 06;9:26-33



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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.

Author List

Nolfi-Donegan D, Pradhan-Sundd T, Pritchard KA Jr, Hillery CA


Kirkwood A. Pritchard PhD Professor in the Surgery department at Medical College of Wisconsin

jenkins-FCD Prod-482 91ad8a360b6da540234915ea01ff80e38bfdb40a