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X Demographics
Mendeley readers
Attention Score in Context
| Title |
Familial autoinflammation with neutrophilic dermatosis reveals a regulatory mechanism of pyrin activation
|
|---|---|
| Published in |
Science Translational Medicine, March 2016
|
| DOI | 10.1126/scitranslmed.aaf1471 |
| Pubmed ID | |
| Authors |
Seth L Masters, Vasiliki Lagou, Isabelle Jéru, Paul J Baker, Lien Van Eyck, David A Parry, Dylan Lawless, Dominic De Nardo, Josselyn E Garcia-Perez, Laura F Dagley, Caroline L Holley, James Dooley, Fiona Moghaddas, Emanuela Pasciuto, Pierre-Yves Jeandel, Raf Sciot, Dena Lyras, Andrew I Webb, Sandra E Nicholson, Lien De Somer, Erika van Nieuwenhove, Julia Ruuth-Praz, Bruno Copin, Emmanuelle Cochet, Myrna Medlej-Hashim, Andre Megarbane, Kate Schroder, Sinisa Savic, An Goris, Serge Amselem, Carine Wouters, Adrian Liston |
| Abstract |
Pyrin responds to pathogen signals and loss of cellular homeostasis by forming an inflammasome complex that drives the cleavage and secretion of interleukin-1β (IL-1β). Mutations in the B30.2/SPRY domain cause pathogen-independent activation of pyrin and are responsible for the autoinflammatory disease familial Mediterranean fever (FMF). We studied a family with a dominantly inherited autoinflammatory disease, distinct from FMF, characterized by childhood-onset recurrent episodes of neutrophilic dermatosis, fever, elevated acute-phase reactants, arthralgia, and myalgia/myositis. The disease was caused by a mutation inMEFV, the gene encoding pyrin (S242R). The mutation results in the loss of a 14-3-3 binding motif at phosphorylated S242, which was not perturbed by FMF mutations in the B30.2/SPRY domain. However, loss of both S242 phosphorylation and 14-3-3 binding was observed for bacterial effectors that activate the pyrin inflammasome, such asClostridiumdifficiletoxin B (TcdB). The S242R mutation thus recapitulated the effect of pathogen sensing, triggering inflammasome activation and IL-1β production. Successful therapy targeting IL-1β has been initiated in one patient, resolving pyrin-associated autoinflammation with neutrophilic dermatosis. This disease provides evidence that a guard-like mechanism of pyrin regulation, originally identified for Nod-like receptors in plant innate immunity, also exists in humans. |
X Demographics
The data shown below were collected from the profiles of 19 X users who shared this research output. Click here to find out more about how the information was compiled.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United States | 4 | 21% |
| France | 1 | 5% |
| Ireland | 1 | 5% |
| Australia | 1 | 5% |
| United Kingdom | 1 | 5% |
| Unknown | 11 | 58% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 17 | 89% |
| Science communicators (journalists, bloggers, editors) | 1 | 5% |
| Scientists | 1 | 5% |
Mendeley readers
The data shown below were compiled from readership statistics for 173 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United Kingdom | 1 | <1% |
| Norway | 1 | <1% |
| Unknown | 171 | 99% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Ph. D. Student | 31 | 18% |
| Researcher | 29 | 17% |
| Other | 16 | 9% |
| Student > Master | 14 | 8% |
| Student > Bachelor | 13 | 8% |
| Other | 37 | 21% |
| Unknown | 33 | 19% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 42 | 24% |
| Biochemistry, Genetics and Molecular Biology | 30 | 17% |
| Agricultural and Biological Sciences | 26 | 15% |
| Immunology and Microbiology | 25 | 14% |
| Neuroscience | 4 | 2% |
| Other | 5 | 3% |
| Unknown | 41 | 24% |
Attention Score in Context
This research output has an Altmetric Attention Score of 62. 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 27 June 2021.
All research outputs
#581,156
of 22,858,915 outputs
Outputs from Science Translational Medicine
#1,467
of 5,108 outputs
Outputs of similar age
#11,749
of 300,631 outputs
Outputs of similar age from Science Translational Medicine
#29
of 123 outputs
Altmetric has tracked 22,858,915 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 97th percentile: it's in the top 5% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 5,108 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 82.0. This one has gotten more attention than average, scoring higher than 71% 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 300,631 tracked outputs that were published within six weeks on either side of this one in any source. This one has done particularly well, scoring higher than 96% of its contemporaries.
We're also able to compare this research output to 123 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 76% of its contemporaries.