Savage C O, Gaskin G, Pusey C D, Pearson J D
Section of Vascular Biology, Royal Postgraduate Medical School, London, UK.
Exp Nephrol. 1993 May-Jun;1(3):190-5.
Anti-neutrophil cytoplasm antibodies (ANCA) are strongly associated with the development of systemic vasculitis. Myeloperoxidase and proteinase-3 have been identified as targets for P-ANCA and C-ANCA, respectively. Both enzymes are released from neutrophil azurophil granules following neutrophil activation and both are highly cationic. Purified myeloperoxidase is demonstrated to bind non-convalently to endothelial cell membranes, to retain its enzymic function following binding, and to retain its antigenicity for P-ANCA. Endothelial cell-bound myeloperoxidase enhances complement-dependent cytotoxicity of some P-ANCA sere that also contain anti-endothelial cell antibodies. Studies using purified proteinase-3 show that it also can bind to endothelial cells and be recognized by C-ANCA. The interactions of myeloperoxidase and proteinase-3 with endothelial cells and ANCA may thus contribute to the development of vascular injury in patients with systemic vasculitis.
抗中性粒细胞胞浆抗体（ANCA）与系统性血管炎的发生密切相关。髓过氧化物酶和蛋白酶-3分别被确定为P-ANCA和C-ANCA的靶抗原。这两种酶在中性粒细胞激活后从中性粒细胞嗜天青颗粒中释放出来，且都带高度正电荷。纯化的髓过氧化物酶被证明能非共价结合到内皮细胞膜上，结合后仍保留其酶活性，并保留其对P-ANCA的抗原性。结合在内皮细胞上的髓过氧化物酶可增强某些同时含有抗内皮细胞抗体的P-ANCA血清的补体依赖性细胞毒性。使用纯化蛋白酶-3的研究表明，它也能结合内皮细胞并被C-ANCA识别。因此，髓过氧化物酶和蛋白酶-3与内皮细胞及ANCA的相互作用可能促使系统性血管炎患者发生血管损伤。
