Taguchi R, Funahashi Y, Ikezawa H, Nakashima I
Faculty of Pharmaceutical Sciences, Nagoya City University, Japan.
FEBS Lett. 1990 Feb 12;261(1):142-6. doi: 10.1016/0014-5793(90)80656-4.
FACS analysis together with PIPLC treatment was applied to PI-anchoring antigens such as DAF (decay-accerelating factor, CD55), 1F5 antigen (CD59), CD14 and CD16 on the cell surfaces of blood cells from a normal adult and a male patient with paroxysmal nocturnal hemoglubinuria (PNH). Through the extensive analysis, this patient proved to be completely defective in 1F5 antigen, a newly found complement-regulatory protein, on all the blood cells tested. In normal blood cells such as lymphocytes, monocytes and granulocytes, 1F5 antigen was expressed as one of PI-anchoring proteins. In contrast to most of PNH patients, this patient reserved DAF, CD14 and CD16 at normal levels in his erythrocytes, monocytes and granulocytes. Also, there were no significant differences between the normal adult and the patient in the activities of erythrocyte acetylcholinesterase and granulocyte alkaline phosphatase which were also known to be PI-anchoring enzymes. Thus, deficiency of 1F5 antigen must be deeply related to the clinical symptoms of PNH in this patient.
将荧光激活细胞分选术（FACS）分析与磷脂酰肌醇特异性磷脂酶C（PIPLC）处理相结合，应用于正常成年人和一名阵发性夜间血红蛋白尿（PNH）男性患者血细胞表面的磷脂酰肌醇（PI）锚定抗原，如衰变加速因子（DAF，CD55）、1F5抗原（CD59）、CD14和CD16。通过广泛分析，该患者被证明在所有检测的血细胞上，一种新发现的补体调节蛋白1F5抗原完全缺失。在正常血细胞如淋巴细胞、单核细胞和粒细胞中，1F5抗原作为PI锚定蛋白之一表达。与大多数PNH患者不同，该患者的红细胞、单核细胞和粒细胞中DAF、CD14和CD16水平正常。此外，正常成年人与该患者的红细胞乙酰胆碱酯酶和粒细胞碱性磷酸酶活性也无显著差异，这两种酶也已知是PI锚定酶。因此，1F5抗原的缺失一定与该患者PNH的临床症状密切相关。
