Seko Y, Yazaki Y
Third Department of Internal Medicine, Faculty of Medicine, University of Tokyo.
Nihon Rinsho. 1994 Aug;52(8):2018-23.
Cell-mediated autoimmunity has been strongly implicated in the pathogenesis of vascular injury in Takayasu's arteritis. Both cytotoxic T lymphocytes (CTLs) and natural killer (NK)cells, playing a major part in cell-mediated cytotoxicity, are thought to kill virus-infected cells or tumor cells with the effector molecules contained in their cytoplasmic granules, one of which is named pore-forming protein or perforin. Perforin is expected to play a central role in the granule exocytosis model of lymphocyte-mediated cytolysis, exhibited by CTLs and NK cells as well as gamma delta T lymphocytes. We found that perforin was expressed in these killer lymphocytes infiltrated in the aortic tissue of patients with Takayasu's arteritis. Immunoelectron microscopic study demonstrated that the infiltrating cells directly injured vascular cells by releasing perforin molecules, indicating the critical role of perforin in the vascular injury involved in Takayasu's arteritis.
细胞介导的自身免疫在大动脉炎血管损伤的发病机制中具有重要作用。细胞毒性T淋巴细胞（CTL）和自然杀伤（NK）细胞在细胞介导的细胞毒性中起主要作用，它们被认为通过其细胞质颗粒中所含的效应分子杀死病毒感染的细胞或肿瘤细胞，其中一种效应分子被称为成孔蛋白或穿孔素。穿孔素有望在淋巴细胞介导的细胞溶解颗粒胞吐模型中发挥核心作用，CTL、NK细胞以及γδT淋巴细胞均表现出这种模型。我们发现，穿孔素在大动脉炎患者主动脉组织中浸润的这些杀伤淋巴细胞中表达。免疫电子显微镜研究表明，浸润细胞通过释放穿孔素分子直接损伤血管细胞，这表明穿孔素在大动脉炎所涉及的血管损伤中起关键作用。
