Critical Care Medicine Department, Clinical Center, National Institutes of Health, Bethesda, MD 20892, USA.
Expert Rev Proteomics. 2010 Dec;7(6):833-48. doi: 10.1586/epr.10.88.
The expanding realm of exploratory proteomics has added a unique dimension to the study of the complex pathophysiology involved in sickle cell disease. A review of proteomic studies published on sickle cell erythrocytes and plasma shows trends of upregulation of antioxidant proteins, an increase in cytoskeletal defects, an increase in protein repair and turnover components, a decrease in lipid raft proteins and apolipoprotein dysregulation. Many of these findings are consistent with the pathophysiology of sickle cell disease, including high oxidant burden, resulting in damage to cytoskeletal and other proteins, and erythrocyte rigidity. More unexpected findings, such as a decrease in lipid raft components and apolipoprotein dysregulation, offer previously unexplored targets for future investigation and potential therapeutic intervention. Exploratory proteomic profiling is a valuable source of hypothesis generation for the cellular and molecular pathophysiology of sickle cell disease.
探索性蛋白质组学的扩展领域为研究镰状细胞病所涉及的复杂病理生理学增加了一个独特的维度。对发表在镰状红细胞和血浆的蛋白质组学研究的综述表明,抗氧化蛋白的上调、细胞骨架缺陷的增加、蛋白质修复和周转成分的增加、脂质筏蛋白和载脂蛋白失调的减少等趋势。这些发现中有许多与镰状细胞病的病理生理学一致,包括高氧化剂负担,导致细胞骨架和其他蛋白质受损,以及红细胞刚性。更意想不到的发现,如脂质筏成分的减少和载脂蛋白失调,为未来的研究和潜在的治疗干预提供了以前未探索的靶点。探索性蛋白质组学分析是镰状细胞病细胞和分子病理生理学假说产生的有价值的来源。