Redox Signaling in Sickle Cell Disease.

Autor: Nolfi-Donegan D; Department of Pediatrics, Division of Pediatric Hematology/Oncology, University of Pittsburgh School of Medicine, Pittsburgh, PA, United States., Pradhan-Sundd T; Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA, United States., Pritchard KA Jr; Department of Surgery, Division of Pediatric Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, United States., Hillery CA; Department of Pediatrics, Division of Pediatric Hematology/Oncology, University of Pittsburgh School of Medicine, Pittsburgh, PA, United States.
Jazyk: angličtina
Zdroj: Current opinion in physiology [Curr Opin Physiol] 2019 Jun; Vol. 9, pp. 26-33. Date of Electronic Publication: 2019 May 02.
DOI: 10.1016/j.cophys.2019.04.022
Abstrakt: 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.
Competing Interests: Declaration of Interests: None
Databáze: MEDLINE
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