CAJ | 학술논문

抗磷脂综合征是一种非炎症性自身免疫疾病,以患者血栓形成和(或)病态妊娠、血中存在抗磷脂抗体为临床特征.β2糖蛋白Ⅰ是该病的主要自身抗原,它的独特的氨基酸序列、具有构想变化和氧化还原修饰等特性使得它在疾病发病机制中占重要作用.它与其自身抗体结合交联触发靶细胞外和胞内信号通路,引起血栓形成和病态妊娠.进一步明确β2糖蛋白Ⅰ与其抗体间相互作用、该抗原抗体复合物引起血液稳态失衡的具体机制有助于开发更有效的针对抗磷脂综合征发病机制的特异性治疗手段.
항린지종합정시일충비염증성자신면역질병,이환자혈전형성화(혹)병태임신、혈중존재항린지항체위림상특정.β2당단백Ⅰ시해병적주요자신항원,타적독특적안기산서렬、구유구상변화화양화환원수식등특성사득타재질병발병궤제중점중요작용.타여기자신항체결합교련촉발파세포외화포내신호통로,인기혈전형성화병태임신.진일보명학β2당단백Ⅰ여기항체간상호작용、해항원항체복합물인기혈액은태실형적구체궤제유조우개발경유효적침대항린지종합정발병궤제적특이성치료수단.
The antiphospholipid syndrome (APS) is a non-inflammatory autoimmune disease clinically characterized by the presence of antiphospholipid antibodies (aPL) in the plasma of patients with vascular thrombosis,pregnancy morbidity,or both.Most of the current evidence indicates that β2glycoprotein Ⅰ (β2GPI) is the major autoantigen in the APS.Its unique amino acid composition and specific properties including conformation change and redox modification indicate the important role of this protein in the pathophysiology of APS.Binding and cross-linking between β2GPI and its autoantibodies trigger intracellular and extracellular pathways that result in thrombosis and may cause pregnancy complications.Future studies on identifying the molecular mechanism for β2GPI interaction with its autoantibodies and the consequences of these interactions for the haemostatic imbalance will potentiate a more effective therapy based on the pathogenesis mechanisms specific to APS.