The Receptor for Advanced Glycation End Products (RAGE) Gene Polymorphisms in Pediatrics with Sickle Cell Disease and Sickle Thalassemia

Abstract

ickle cell disease (SCD) induces chronic states of oxidative stress and inflammation. There is an increasing evidence points towards an oxidative stress response responsible for the pathophysiology of secondary dysfunction in sickle cell patients. Oxidative stress results in increased production of AGEs which cause a wide range of deleterious effects mediated by their receptors RAGE. Upon AGE-RAGE binding, RAGE activates multiple downstream cellular signaling cascade resulting in sustained upregulation of pro-inflammatory mediators and oxidants resulting in a dysfunctional cell phenotype. Formation of AGE and the consequences of AGE/RAGE interaction have been studied in SCD, previously.