Perforin deficiency and susceptibility to cancer

被引：50

作者：

Brennan, A. J. ^{[1
,2
]}

Chia, J. ^{[1
]}

Trapani, J. A. ^{[1
,3
]}

Voskoboinik, I. ^{[1
,4
]}

机构：

[1] Peter MacCallum Canc Ctr, Canc Immunol Program, Melbourne, Vic 3002, Australia

[2] Univ Melbourne, Dept Pathol, Parkville, Vic 3052, Australia

[3] Univ Melbourne, Dept Microbiol & Immunol, Parkville, Vic 3052, Australia

[4] Univ Melbourne, Dept Genet, Parkville, Vic 3052, Australia

来源：

CELL DEATH AND DIFFERENTIATION | 2010年 / 17卷 / 04期

关键词：

cytotoxic lymphocytes; lymphoma; leukemia; granzyme; FHL; FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; GRANZYME-B INHIBITOR; NATURAL-KILLER-CELLS; PORE-FORMING PROTEIN; CD8(+) T-CELLS; MEDIATED APOPTOSIS; HUMAN KERATINOCYTES; A91V POLYMORPHISM; INTERFERON-GAMMA; GENE-MUTATIONS;

D O I：

10.1038/cdd.2009.212

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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. Cell Death and Differentiation (2010) 17, 607-615; doi: 10.1038/cdd.2009.212; published online 15 January 2010

引用

页码：607 / 615

页数：9

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