Zeta Perforin Antibody. Zeta’s rabbit recombinant antibody recognizes Perforin, a glycoprotein responsible for pore formation in cell membranes, where Perforin is able to polymerize and form a channel in the cell membrane. Polymerized Perforin molecules form channels enabling free, non-selective, passive transport of ions, water, small-molecule substances and enzymes.
Perforin, a protein found within cytoplasmic granules, is a marker of cytotoxic lymphocytes. It is frequently positive, along with granzyme B, in extra-nodal natural killer (NK) cell lymphomas, aggressive NK cell leukemias and large granular lymphocyte (LGL) leukemias. Unlike TIA1, it is negative in hepatosplenic T-cell lymphoma.
As a pore-forming protein, Perforin can lead to osmotic lysis and subsequently enables granzymes to enter the cells and activate apoptosis. 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. Perforin 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 lympho-histiocytosis type 2 (HPLH2), a rare and lethal autosomal recessive disorder of early childhood. Abnormal Perforin production plays a role in other diseases including leukemias and lymphomas, infectious diseases and autoimmune diseases, immune response to bacterial and viral infections, immune surveillance and immunopathology.
Perforin is involved in the pathogenesis of autoimmune diseases and allogeneic transplant rejection. The expression of Perforin is reportedly upregulated in activated CD8+ T-cells, natural killer cells and some CD4+ T-cells. Additionally, CD4-positive T-cells express a low amount of Perforin, when classic cytotoxicity is ineffective or disturbed.
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