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- Title
Perforin deficiency and susceptibility to cancer.
- Authors
Brennan, A J; Chia, J; Trapani, J A; Voskoboinik, I
- Abstract
Cytotoxic lymphocytes (CLs) are the killer cells that destroy intracellular pathogen-infected and transformed cells, predominantly through the cytotoxic granule-mediated death pathway. Soluble cytotoxic granule components, including pore-forming perforin and pro-apoptotic serine proteases, granzymes, synergize to induce unscheduled apoptosis of the target cell. A complete loss of CL function results in an aggressive immunoregulatory disorder, familial hemophagocytic lymphohistiocytosis, whereas a partial loss of function seems to be a factor strongly predisposing to hematological malignancies. This review discusses the pathological manifestations of CL deficiencies due to impaired perforin function and describes novel aspects of perforin biology.
- Publication
Cell death and differentiation, 2010, Vol 17, Issue 4, p607
- ISSN
1476-5403
- Publication type
Journal Article
- DOI
10.1038/cdd.2009.212