نتایج جستجو برای: perforin gene

تعداد نتایج: 1142550  

2013
Hyun Yang Young Mee Lee Jeong-Ho Lee Jae Koo Noh Hyun Chul Kim Choul-Ji Park Jong-Won Park In Joon Hwang Sung Yeon Kim

The innate immune system is the only defense weapon that invertebrates have, and it is the fundamental defense mechanism for fish. The innate immune response is important in newly hatched flounders because it is closely involved in the initial feeding phase, which is why it is essential for survival during the juvenile period. The expression analysis of genes involved in the innate immune respo...

Journal: :The Journal of Experimental Medicine 2004
Shayna E.A. Street Yoshihiro Hayakawa Yifan Zhan Andrew M. Lew Duncan MacGregor Amanda M. Jamieson Andreas Diefenbach Hideo Yagita Dale I. Godfrey Mark J. Smyth

Few studies have demonstrated that innate lymphocytes play a major role in preventing spontaneous tumor formation. We evaluated the development of spontaneous tumors in mice lacking beta-2 microglobulin (beta2m; and thus MHC class I, CD1d, and CD16) and/or perforin, since these tumor cells would be expected to activate innate effector cells. Approximately half the cohort of perforin gene-target...

2018
Robin C Willenbring Yasuhiro Ikeda Larry R Pease Aaron J Johnson

BACKGROUND Deleterious mutations in PRF1 result in lethal, childhood disease, familial hemophagocytic lymphohistiocytosis type 2 (FHL 2). However, not all mutations in PRF1 are deleterious and result in FHL 2. Currently, these nondeleterious mutations are being investigated in the onset of numerous disorders, such as lymphomas and diabetes. Yet, there is still an overwhelmingly large amount of ...

2011
Rim El Abed Violaine Bourdon Ilia Voskoboinik Halima Omri Yosra Ben Youssef Mohamed Adnene Laatiri Laetitia Huiart François Eisinger Laetitia Rabayrol Marc Frenay Paul Gesta Liliane Demange Hélène Dreyfus Valérie Bonadona Catherine Dugast Hélène Zattara Laurence Faivre Monia Zaier Saloua Yacoub Jemni Testsuro Noguchi Hagay Sobol Zohra Soua

Perforin gene (PRF1) mutations have been identified in some patients diagnosed with the familial form of hemophagocytic lymphohistiocytosis (HLH) and in patients with lymphoma. The aim of the present study was to determine whether patients with a familial aggregation of hematological malignancies harbor germline perforin gene mutations. For this purpose, 81 unrelated families from Tunisia and F...

Journal: :archives of pediatric infectious diseases 0
roxana m. ghanaie 1 pediatric infections research center, shahid beheshti university of medical sciences, tehran, ir iranسازمان اصلی تایید شده: دانشگاه علوم پزشکی شهید بهشتی (shahid beheshti university of medical sciences) reza shiari department of pediatrics, division of pediatric rheumatology, mofid children hospital, shahid beheshti university of medical sciences, ir iranسازمان اصلی تایید شده: دانشگاه علوم پزشکی شهید بهشتی (shahid beheshti university of medical sciences) abdollah karimi 1 pediatric infections research center, shahid beheshti university of medical sciences, tehran, ir iranسازمان اصلی تایید شده: دانشگاه علوم پزشکی شهید بهشتی (shahid beheshti university of medical sciences) shahnaz armin 1 pediatric infections research center, shahid beheshti university of medical sciences, tehran, ir iranسازمان اصلی تایید شده: دانشگاه علوم پزشکی شهید بهشتی (shahid beheshti university of medical sciences) alireza fahimzad 1 pediatric infections research center, shahid beheshti university of medical sciences, tehran, ir iranسازمان اصلی تایید شده: دانشگاه علوم پزشکی شهید بهشتی (shahid beheshti university of medical sciences) faride shiva 1 pediatric infections research center, shahid beheshti university of medical sciences, tehran, ir iranسازمان اصلی تایید شده: دانشگاه علوم پزشکی شهید بهشتی (shahid beheshti university of medical sciences)

hemophagocytic lymphohistiocytosis (hlh) is an aggressive and potentially life-threatening disease and has to be considered in the differential diagnosis of many conditions. hlh comprises two different conditions that are difficult to differentiate; familial hemophagocytic lymphohistiocytosis (fhlh) or familial erythrophagocytic lymphohistiocytosis (fel), and secondary hemophagocytic syndromes ...

Journal: :Journal of immunology 2010
Montse Camiña-Tato Carlos Morcillo-Suárez Marta F Bustamante Israel Ortega Arcadi Navarro Aura Muntasell Miguel López-Botet Alex Sánchez Paco Carmona Eva Julià María Teresa Tortola Laura Audí Jorge R Oksenberg Roland Martin Xavier Montalban Manuel Comabella

The granule-dependent exocytosis pathway is an important mechanism to induce apoptosis by CD8(+) T cells and NK cells and involves lytic molecules such as perforin. In the current study, we investigated the perforin 1 gene (PRF1) as a candidate for multiple sclerosis (MS) susceptibility in the Spanish population. We genotyped three PRF1 single nucleotide polymorphisms (rs885822, rs10999426, and...

2013
Raymond M. Johnson Micah S. Kerr James E. Slaven

CD4 T cells are critical for clearing experimental Chlamydia muridarum genital tract infections. Two independent in vitro CD4 T cell mechanisms have been identified for terminating Chlamydia replication in epithelial cells. One mechanism, requiring IFN-γ and T cell-epithelial cell contact, terminates infection by triggering epithelial production of nitric oxide to chlamydiacidal levels; the sec...

Background: Perforin is known to be important in cytolytic activity mediated by natural killer (NK) cells.   Objective: To study the relationship between the efficiency of NK and lymphokine-activated killer (LAK) cells activity, and the expression of perforin and HLA class I molecules.   Methods: LAK cells were generated by in vitro culturing of human peripheral blood lymphocytes (PBLs) in the ...

1996
Bernhard Odermatt Pamela S. Ohashi Hans Hengartner

It is widely accepted that T cells play an important role in the destruction of [3 cells leading to autoimmune type I diabetes, but the involved effector mechanisms have remained unclear. We addressed this issue by testing the role of perforin-dependent cytotoxicity in a disease model involving transgenic mice expressing glycoprotein of lymphocytic choriomeningitis virus (LCMV-GP) in the 13 cel...

Journal: :Blood 1997
S Shresta J H Russell T J Ley

Using granzyme B-deficient mice obtained by gene targeting, we previously demonstrated that granzyme B is required for the rapid induction of apoptotic target cell death by cytotoxic T lymphocytes (CTLs); however, CTLs are also equipped with additional effector mechanisms. In the present study, we examined the mechanisms responsible for granzyme B-independent cytotoxicity using in vitro lytic a...

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