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IUBMB Life. 2012 Jan;64(1):72-80. doi: 10.1002/iub.584. Epub 2011 Nov 30.

Role of oxidative stress in the pathogenesis of sickle cell disease.

Author information

1
Université Claude Bernard Lyon 1, Centre de Recherche et d'Innovation sur le Sport, Université de Lyon, 69622 Villeurbanne Cedex, France.

Abstract

Sickle cell disease (SCD) is a class of hemoglobinopathy in humans, which causes a disruption of the normal activities in different systems. Although this disease begins with the polymerization of red blood cells during its deoxygenating phase, it can erupt into a cascade of debilitating conditions such as ischemia-reperfusion injury, inflammation, and painful vaso-occlusion crises. The purpose of this review is to discuss how these phenomena can result in the formation of oxidative stress as well as limit nitric oxide (NO) bioavailability and decrease antioxidant status. The cumulative effects of these traits cause an increase in other forms of reactive oxygen species (ROS), which in turn intensify the symptoms of SCD and generate a vicious circle. Finally, we will discuss antioxidant therapeutic strategies that limit ROS generation and subsequently increase NO bioavailability with respect to endothelial protection in SCD.

PMID:
22131167
DOI:
10.1002/iub.584
[Indexed for MEDLINE]
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