Natural Killer Cells from Patients with Recombinase-Activating Gene and Non-Homologous End Joining Gene Defects Comprise a Higher Frequency of CD56bright NKG2A+++ Cells, and Yet Display Increased Degranulation and Higher Perforin Content

Natural Killer Cells from Patients with Recombinase-Activating Gene and Non-Homologous End Joining Gene Defects Comprise a Higher Frequency of CD56bright NKG2A+++ Cells, and Yet Display Increased Degranulation and Higher Perforin Content

Mutations of the recombinase-activating genes 1 and 2 (RAG1 and RAG2) in humans are associated with a broad range of phenotypes. For patients with severe clinical presentation, hematopoietic stem cell transplantation (HSCT) represents the only curative treatment; however, high rates of graft failure...

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Main Authors: Kerry Dobbs, Giovanna Tabellini, Enrica Calzoni, Ornella Patrizi, Paula Martinez, Silvia Clara Giliani, Daniele Moratto, Waleed Al-Herz, Caterina Cancrini, Morton Cowan, Jacob Bleesing, Claire Booth, David Buchbinder, Siobhan O. Burns, Talal A. Chatila, Janet Chou, Vanessa Daza-Cajigal, Lisa M. Ott de Bruin, Maite Teresa de la Morena, Gigliola Di Matteo, Andrea Finocchi, Raif Geha, Rakesh K. Goyal, Anthony Hayward, Steven Holland, Chiung-Hui Huang, Maria G. Kanariou, Alejandra King, Blanka Kaplan, Anastasiya Kleva, Taco W. Kuijpers, Bee Wah Lee, Vassilios Lougaris, Michel Massaad, Isabelle Meyts, Megan Morsheimer, Benedicte Neven, Sung-Yun Pai, Nima Parvaneh, Alessandro Plebani, Susan Prockop, Ismail Reisli, Jian Yi Soh, Raz Somech, Troy R. Torgerson, Yae-Jaen Kim, Jolan E. Walter, Andrew R. Gennery, Sevgi Keles, John P. Manis, Emanuela Marcenaro, Alessandro Moretta, Silvia Parolini, Luigi D. Notarangelo
Format: Article
Language:English
Published: Frontiers Media S.A. 2017-07-01
Series:Frontiers in Immunology
Subjects:
natural killer cells
recombinase-activating genes
non-homologous end joining
immunodeficiency
CD56
interferon-γ
Online Access:http://journal.frontiersin.org/article/10.3389/fimmu.2017.00798/full

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http://journal.frontiersin.org/article/10.3389/fimmu.2017.00798/full

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