Thiery Jerome, Lieberman Judy
INSERM U753, University Paris Sud and Gustave Roussy Cancer Campus, Villejuif, France,
Subcell Biochem. 2014;80:197-220. doi: 10.1007/978-94-017-8881-6_10.
Perforin (PFN) is the key pore-forming molecule in the cytotoxic granules of immune killer cells. Expressed only in killer cells, PFN is the rate-limiting molecule for cytotoxic function, delivering the death-inducing granule serine proteases (granzymes) into target cells marked for immune elimination. In this chapter we describe our current understanding of how PFN accomplishes this task. We discuss where PFN is expressed and how its expression is regulated, the biogenesis and storage of PFN in killer cells and how they are protected from potential damage, how it is released, how it delivers Granzymes into target cells and the consequences of PFN deficiency.
穿孔素（PFN）是免疫杀伤细胞细胞毒性颗粒中的关键成孔分子。PFN仅在杀伤细胞中表达，是细胞毒性功能的限速分子，可将诱导死亡的颗粒丝氨酸蛋白酶（颗粒酶）传递到被标记进行免疫清除的靶细胞中。在本章中，我们描述了目前对PFN如何完成这项任务的理解。我们讨论了PFN在哪里表达以及其表达如何被调节，PFN在杀伤细胞中的生物合成和储存以及它们如何免受潜在损伤，它如何释放，如何将颗粒酶传递到靶细胞中以及PFN缺乏的后果。
