Anti-Perforin-1 (Pore Forming Protein) (Apoptosis Marker) Monoclonal Antibody (Clone: PRF1/2470)

Perforin is a pore-forming protein that leads to osmotic lysis of the target cells and subsequently enables granzymes to enter the target cells and activate apoptosis. Perforin has structural and functional similarities to complement component 9 (C9). Like C9, this protein 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 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. The expression of perforin is reportedly upregulated in activated CD8+ T-cells, natural killer cells and some CD4+ T-cells. Protein function: Pore-forming protein that plays a key role in secretory granule-dependent cell death, and in defense against virus-infected or neoplastic cells (PubMed:9058810, PubMed:9164947, PubMed:20889983, PubMed:21037563). Plays an important role in killing other cells that are recognized as non-self by the immune system, e.g. in transplant rejection or some forms of autoimmune disease (PubMed:9058810). Can insert into the membrane of target cells in its calcium-bound form, oligomerize and form large pores (PubMed:20889983, PubMed:21037563). Promotes cytolysis and apoptosis of target cells by facilitating the uptake of cytotoxic granzymes (PubMed:20038786, PubMed:20225066, PubMed:32299851). [The UniProt Consortium]