Weinstock Christof
German Red Cross Blood Service Baden-Württemberg - Hessen, Institute Ulm, Ulm, Germany.
Institute for Clinical Transfusion Medicine and Immunogenetics Ulm, Ulm, Germany.
Transfus Med Hemother. 2022 Jan 13;49(1):13-24. doi: 10.1159/000521174. eCollection 2022 Feb.
In 2014, the membrane-bound protein CD59 became a blood group antigen. CD59 has been known for decades as an inhibitor of the complement system, located on erythrocytes and on many other cell types. In paroxysmal nocturnal haemoglobinuria (PNH), a stem cell clone with acquired deficiency to express GPI-anchored molecules, including the complement inhibitor CD59, causes severe and life-threatening disease. The lack of CD59, which is the only membrane-bound inhibitor of the membrane attack complex, contributes a major part of the intravascular haemolysis observed in PNH patients. This crucial effect of CD59 in PNH disease prompted studies to investigate its role in other diseases. In this review, the role of CD59 in inflammation, rheumatic disease, and age-related macular degeneration is investigated. Further, the pivotal role of CD59 in PNH and congenital CD59 deficiency is reviewed.
2014年,膜结合蛋白CD59成为一种血型抗原。几十年来,CD59一直作为补体系统的抑制剂为人所知,它存在于红细胞及许多其他细胞类型上。在阵发性夜间血红蛋白尿(PNH)中,一个获得性表达糖基磷脂酰肌醇(GPI)锚定分子缺陷的干细胞克隆,包括补体抑制剂CD59,会引发严重的危及生命的疾病。CD59是膜攻击复合物唯一的膜结合抑制剂,其缺失是PNH患者血管内溶血的主要原因。CD59在PNH疾病中的这一关键作用促使人们开展研究以探究其在其他疾病中的作用。在本综述中,我们研究了CD59在炎症、风湿性疾病和年龄相关性黄斑变性中的作用。此外,还综述了CD59在PNH和先天性CD59缺乏症中的关键作用。