Oxidative alterations in sickle cell disease: Possible involvement in disease pathogenesis  

作　　者：Yesim Oztas Ahmet Yalcinkaya 

机构地区：[1]Department of Medical Biochemistry, Faculty of Medicine, Hacettepe University

出　　处：《World Journal of Hematology》2017年第3期55-61,共7页世界血液学杂志

摘　　要：Sickle cell disease(SCD) is the first molecular disease in the literature. Although the structural alteration and dysfunction of the sickle hemoglobin(HbS) are well understood, the many factors modifying the clinical signs and symptoms of the disease are under investigation. Besides having an abnormal electrophoretic mobility and solubility, HbS is unstable. The autooxidation rate of the abnormal HbS has been reported to be almost two times of the normal. There are two more components of the oxidative damage in SCD: Free radical induced oxidative damage during vaso-occlusion induced ischemia-reperfusion injury and decreased antioxidant capacity in the erythrocyte and in the circulation. We will discuss the effects of oxidative alterations in the erythrocyte and in the plasma of SCD patients in this review.Sickle cell disease(SCD) is the first molecular disease in the literature. Although the structural alteration and dysfunction of the sickle hemoglobin(HbS) are well understood, the many factors modifying the clinical signs and symptoms of the disease are under investigation. Besides having an abnormal electrophoretic mobility and solubility, HbS is unstable. The autooxidation rate of the abnormal HbS has been reported to be almost two times of the normal. There are two more components of the oxidative damage in SCD: Free radical induced oxidative damage during vaso-occlusion induced ischemia-reperfusion injury and decreased antioxidant capacity in the erythrocyte and in the circulation. We will discuss the effects of oxidative alterations in the erythrocyte and in the plasma of SCD patients in this review.

关 键 词：OXIDATIVE stress SICKLE cell DISEASE Iron Protein oxidation Carbonyl GROUP SULFHYDRYL GROUP Low-density LIPOPROTEIN High-density LIPOPROTEIN 

分 类 号：R[医药卫生]