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

Date

06/27/2019

Pubmed ID

31240269

Pubmed Central ID

PMC6592428

DOI

10.1016/j.cophys.2019.04.022

Abstract

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

Author

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




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