Perforin

Perforin has structural and functional similarities to complement component 9 (C9). Like C9, Perforin creates transmembrane tubules and is capable of lysing non-specifically a variety of target cells. It is one of the main cytolytic proteins of cytolytic granules, and it is known to be a key effector molecule for T-cell- and natural killer-cell-mediated cytolysis. Defects in this gene cause familial hemophagocytic lymphohistiocytosis type 2 (HPLH2), a rare and lethal autosomal recessive disorder of early childhood.