Telen M J, Rosse W F
Baillieres Clin Haematol. 1991 Dec;4(4):849-68. doi: 10.1016/s0950-3536(06)80033-8.
The human erythrocyte bears a number of proteins anchored to the outer membrane surface via a phosphatidylinositol-glycan linkage. This class of proteins includes several complement regulatory proteins (including decay-accelerating factor, CD59 antigen (protectin), and C8 binding protein) as well as several enzymes and at least one protein important in cell-cell interaction. In addition, a number of blood group antigens have been identified to reside on proteins with phosphatidylinositol anchors. One blood group (Cromer) resides on DAF. Study of variants in this blood group system has led to interesting information about the function and expression of this protein. Several other blood groups, such as JMH and Holley/Gregory, appear to reside on as yet unidentified phosphatidylinositol-linked proteins. In paroxysmal nocturnal haemoglobinuria, a variable proportion of red cells fail to express or express weakly all phosphatidylinositol-linked proteins. The origin of this deficiency is now being worked out. In addition, individuals with inherited deficiency of DAF or CD59 (protectin) have been identified. Only the latter deficiency leads to a PNH-like syndrome.
人类红细胞带有多种通过磷脂酰肌醇 - 聚糖连接锚定在外膜表面的蛋白质。这类蛋白质包括几种补体调节蛋白（包括衰变加速因子、CD59抗原（保护素）和C8结合蛋白）以及几种酶和至少一种在细胞间相互作用中起重要作用的蛋白质。此外，已确定多种血型抗原存在于带有磷脂酰肌醇锚的蛋白质上。一种血型（克勒默血型）存在于衰变加速因子上。对该血型系统变体的研究得出了有关这种蛋白质功能和表达的有趣信息。其他几种血型，如JMH和霍利/格雷戈里血型，似乎存在于尚未鉴定的磷脂酰肌醇连接蛋白上。在阵发性夜间血红蛋白尿中，可变比例的红细胞无法表达或微弱表达所有磷脂酰肌醇连接蛋白。这种缺陷的起源目前正在研究中。此外，已鉴定出遗传性衰变加速因子或CD59（保护素）缺乏的个体。只有后者的缺乏会导致类似阵发性夜间血红蛋白尿的综合征。
