Charles L A, Caldas M L, Falk R J, Terrell R S, Jennette J C
Department of Pathology, University of North Carolina School of Medicine, Chapel Hill 27599-7525.
J Leukoc Biol. 1991 Dec;50(6):539-46. doi: 10.1002/jlb.50.6.539.
Polymorphonuclear leukocyte (PMN) respiratory burst was stimulated by heterologous antibodies against PMN granule proteins but not by control antibodies. Fluorescence-activated cell sorter (FACS) analysis of activated PMN demonstrated the presence of two primary granule proteins, proteinase 3 (PR-3) and cationic protein 57 (CAP-57) at the membrane surface. The presence of myeloperoxidase (MPO) at the cell surface of primed and unprimed PMN was confirmed by immunoelectron microscopy. Priming doses of recombinant tumor necrosis alpha (rTNF alpha) enhanced the rate of superoxide (O2-) production by these antibodies and increased the amount of surface protein accessible to these antibodies. Anti-neutrophil cytoplasmic autoantibodies (ANCA) with specificities for PMN granule proteins are present in patients with Wegener's granulomatosis, polyarteritis nodosa, and idiopathic and crescentic glomerulonephritis. The demonstration that antibodies against granule proteins activate PMN supports the hypothesis that the vasculitis seen in these diseases is due in part to PMN mediated oxidative injury following PMN stimulation by ANCA.
抗多形核白细胞（PMN）颗粒蛋白的异种抗体可刺激PMN呼吸爆发，而对照抗体则无此作用。对活化的PMN进行荧光激活细胞分选仪（FACS）分析显示，膜表面存在两种主要颗粒蛋白，即蛋白酶3（PR-3）和阳离子蛋白57（CAP-57）。免疫电子显微镜证实了致敏和未致敏PMN细胞表面存在髓过氧化物酶（MPO）。重组肿瘤坏死因子α（rTNFα）的致敏剂量提高了这些抗体产生超氧化物（O2-）的速率，并增加了这些抗体可接触到的表面蛋白量。患有韦格纳肉芽肿、结节性多动脉炎、特发性和新月形肾小球肾炎的患者体内存在对PMN颗粒蛋白具有特异性的抗中性粒细胞胞浆自身抗体（ANCA）。针对颗粒蛋白的抗体可激活PMN，这一发现支持了以下假说：这些疾病中出现的血管炎部分归因于ANCA刺激PMN后PMN介导的氧化损伤。
