背景
|
Perforin is a pore forming, cytolytic protein that is a component of cytolytic T-cell and NK cell granules. Perforin activates clathrin- and dynamin-dependent endocytosis, which removes perforin and granzymes from the plasm membrane to early endosomes, preserving outer membrane integrity. Perforin facilitates granzyme uptake and avoids the proinflammatory necrotic death of a membrane-damaged cell. Defects in the Perforin gene results in Familial hemophagocytic lymphohistiocytosis (FHL). Perforin-based effector systems are involved not only in the lysis of abnormal cells but also in the down-regulation of cellular immune activation.
|