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2014 ; 39
(1
): 109-15
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Perforin: an important player in immune response
#MMPMID26155110
Osi?ska I
; Popko K
; Demkow U
Cent Eur J Immunol
2014[]; 39
(1
): 109-15
PMID26155110
show ga
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.
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