title: Perforin: an important player in immune response.
abstract: Perforin is a glycoprotein responsible for pore formation in cell membranes of target cells. Perforin is able to polymerize and form a channel in target cell membrane. Many research groups focus on the role of perforin in various diseases, immune response to bacterial and viral infections, immune surveillance and immunopathology. In addition, perforin is involved in the pathogenesis of autoimmune diseases and allogeneic transplant rejection. Natural killer (NK) cells and CD8-positive T-cells are the main source of perforin. However, CD4-positive T-cells are also able to express a low amount of perforin, when classic cytotoxicity is ineffective or disturbed. Polymerized perforin molecules form channels enabling free, non-selective, passive transport of ions, water, small-molecule substances and enzymes. In consequence, the channels disrupt protective barrier of cell membrane and destroy integrity of the target cell. This review will focus on mechanisms of action and structure of perforin. Also, in this review we discuss the problem of abnormal perforin production in diseases such as: hemophagocytic lymphohistiocytosis (HLH), leukemias and lymphomas, infectious diseases and autoimmune diseases. Better understanding of the role of these molecules in health and disease will open a new field of research with possible therapeutic implications.
authors.affiliation: PhD Study, Department of Pathology, Medical University of Warsaw, Poland ; Department of Laboratory Diagnostics and Clinical Immunology of Developmental Age, Medical University of Warsaw, Poland.
authors.forename: Iwona
authors.initials: I
authors.lastname: Osińska
authors.affiliation: Department of Laboratory Diagnostics and Clinical Immunology of Developmental Age, Medical University of Warsaw, Poland.
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Popko
authors.affiliation: Department of Laboratory Diagnostics and Clinical Immunology of Developmental Age, Medical University of Warsaw, Poland.
authors.forename: Urszula
authors.initials: U
authors.lastname: Demkow
keywords: HLH; Hashimoto's disease; SLE; autoimmune diseases; cytolytic granules; granzyme; type 1 diabetes