Interplay Between Superoxide and Nitric Oxide in Thalassemia and Fanconi's Anemia

Abstract

Thalassemia (thal) and Fanconi's Anemia (FA) are genetic disorders associated with iron-catalyzed free radical damage. Therefore, the contemporary and most successful treatment of thalassemic patients depends on the application of iron (Fe) chelators. However, there is another pathway of free radical-mediated damaging processes in these pathologies, depending on the interplay between physiological free radicals superoxide and nitric oxide (NO). In the present study, we have considered the major routes of superoxide damaging effects in mitochondria: the initiation of apoptosis through the reduction of cytochrome c, the activation of uncoupled proteins by superoxide, and the mitochondrial damage due to the competition between superoxide and nitric oxide at the Complex IV site (cytochrome oxidase). The application of the effective scavengers superoxide dismutases and flavonoids for the treatment of thalassemic and FA patients, is discussed.

Keywords:

• Superoxide
• Nitric oxide
• Thalassemia (thal)
• Fanconi's Anemia (FA)