Warning: Undefined variable $zfal in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 525
Deprecated: str_replace(): Passing null to parameter #3 ($subject) of type array|string is deprecated in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 525
Warning: Undefined variable $sterm in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 530
Warning: Undefined variable $sterm in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 531
English Wikipedia
Nephropedia Template TP (
Twit Text
DeepDyve Pubget Overpricing |
lüll Perforin deficiency and susceptibility to cancer Brennan AJ; Chia J; Trapani JA; Voskoboinik ICell Death Differ 2010[Apr]; 17 (4): 607-15Cytotoxic 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.|Animals[MESH]|Apoptosis Regulatory Proteins/genetics/*metabolism[MESH]|Genetic Predisposition to Disease/genetics[MESH]|Granzymes/metabolism[MESH]|Humans[MESH]|Immune System/enzymology/physiopathology[MESH]|Immunologic Surveillance/*physiology[MESH]|Lymphohistiocytosis, Hemophagocytic/enzymology/genetics/immunology[MESH]|Mice[MESH]|Neoplasms/*enzymology/genetics/*immunology[MESH]|Perforin[MESH]|Pore Forming Cytotoxic Proteins/*deficiency/genetics[MESH]|T-Lymphocytes, Cytotoxic/*enzymology/immunology[MESH] |