葡萄糖磷脂酰肌醇锚定蛋白与阵发性睡眠性血红蛋白尿症发病机制关系的研究进展

Relationship between glucose phosphatidylinositol anchor proteins and the pathogenesis of PNH

阵发性睡眠性血红蛋白尿症(PNH)是一种良性克隆性疾病,该病以1个或多个葡萄糖磷脂酰肌醇(glycosyl phosphatidyl inositol,GPI)锚定蛋白(GPI-APs)缺失或减少的造血细胞克隆的扩增为特征,临床主要表现为血管内溶血、骨髓衰竭和血栓形成。GPI-锚定蛋白的缺失是PNH发病机制中重要一环。文章将从PNH的认识历程、锚蛋白的生物合成、功能及其与PNH发病机制的关系做一综述。

Paroxymal noctural hemoglobinuria(PNH)is a kind of A benign clonal disease,characterized by expansion of hematopoietic cell clones with loss or decrease of glucosyphosphatidylinositol(GPI)anchor proteins(GPI-APs).The main clinical manifestations were intravascular hemolysis,bone marrow failure and thrombosis.Abnormal of GPI-APs played an important role in the pathogenesis of PNH.Therefore,this review will focus on the understanding history,biosynthesis and function of some GPI-APs and its relationship with nosogenesis of PNH.