Cancer Immunology Program, Peter MacCallum Cancer Centre, East Melbourne, Victoria 3002, Australia.
Cell Death Differ. 2010 Apr;17(4):607-15. doi: 10.1038/cdd.2009.212. Epub 2010 Jan 15.
Cytotoxic lymphocytes (CLs) are the killer cells that destroy intracellular pathogen-infected and transformed cells, predominantly through the cytotoxic granule-mediated death pathway. Soluble cytotoxic granule components, including pore-forming perforin and pro-apoptotic serine proteases, granzymes, synergize to induce unscheduled apoptosis of the target cell. A complete loss of CL function results in an aggressive immunoregulatory disorder, familial hemophagocytic lymphohistiocytosis, whereas a partial loss of function seems to be a factor strongly predisposing to hematological malignancies. This review discusses the pathological manifestations of CL deficiencies due to impaired perforin function and describes novel aspects of perforin biology.
细胞毒性淋巴细胞（CLs）是破坏细胞内受病原体感染和转化的细胞的杀伤细胞，主要通过细胞毒性颗粒介导的死亡途径。可溶性细胞毒性颗粒成分，包括形成孔的穿孔素和促凋亡丝氨酸蛋白酶颗粒酶，协同诱导靶细胞的非计划性细胞凋亡。CL 功能完全丧失会导致侵袭性免疫调节紊乱，即家族性噬血细胞性淋巴组织细胞增生症，而部分功能丧失似乎是强烈导致血液恶性肿瘤的一个因素。本文讨论了由于穿孔素功能障碍导致 CL 缺陷的病理表现，并描述了穿孔素生物学的新方面。
