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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…

Overview of attention for article published in Frontiers in immunology, July 2017
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  • Good Attention Score compared to outputs of the same age (67th percentile)
  • Good Attention Score compared to outputs of the same age and source (70th percentile)

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Title
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
Published in
Frontiers in immunology, July 2017
DOI 10.3389/fimmu.2017.00798
Pubmed ID
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

Abstract

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 and incomplete immune reconstitution have been observed, especially after unconditioned haploidentical transplantation. Studies in mice have shown that Rag(-/-) natural killer (NK) cells have a mature phenotype, reduced fitness, and increased cytotoxicity. We aimed to analyze NK cell phenotype and function in patients with mutations in RAG and in non-homologous end joining (NHEJ) genes. Here, we provide evidence that NK cells from these patients have an immature phenotype, with significant expansion of CD56(bright) CD16(-/int) CD57(-) cells, yet increased degranulation and high perforin content. Correlation was observed between in vitro recombinase activity of the mutant proteins, NK cell abnormalities, and in vivo clinical phenotype. Addition of serotherapy in the conditioning regimen, with the aim of depleting the autologous NK cell compartment, may be important to facilitate engraftment and immune reconstitution in patients with RAG and NHEJ defects treated by HSCT.

X Demographics

X Demographics

The data shown below were collected from the profiles of 9 X users who shared this research output. Click here to find out more about how the information was compiled.
Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 44 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 44 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 7 16%
Other 5 11%
Student > Ph. D. Student 4 9%
Professor 3 7%
Professor > Associate Professor 3 7%
Other 6 14%
Unknown 16 36%
Readers by discipline Count As %
Medicine and Dentistry 9 20%
Immunology and Microbiology 9 20%
Biochemistry, Genetics and Molecular Biology 3 7%
Agricultural and Biological Sciences 2 5%
Arts and Humanities 1 2%
Other 1 2%
Unknown 19 43%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 5. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 16 August 2017.
All research outputs
#7,078,644
of 25,461,852 outputs
Outputs from Frontiers in immunology
#7,805
of 31,698 outputs
Outputs of similar age
#98,543
of 307,614 outputs
Outputs of similar age from Frontiers in immunology
#121
of 412 outputs
Altmetric has tracked 25,461,852 research outputs across all sources so far. This one has received more attention than most of these and is in the 71st percentile.
So far Altmetric has tracked 31,698 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 8.4. This one has gotten more attention than average, scoring higher than 74% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 307,614 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 67% of its contemporaries.
We're also able to compare this research output to 412 others from the same source and published within six weeks on either side of this one. This one has gotten more attention than average, scoring higher than 70% of its contemporaries.