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Genomic instability and myelodysplasia with monosomy 7 consequent to EVI1 activation after gene therapy for chronic granulomatous disease

Overview of attention for article published in Nature Medicine, January 2010
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About this Attention Score

  • In the top 25% of all research outputs scored by Altmetric
  • High Attention Score compared to outputs of the same age (96th percentile)
  • High Attention Score compared to outputs of the same age and source (87th percentile)

Mentioned by

blogs
2 blogs
policy
1 policy source
twitter
1 X user
patent
11 patents
wikipedia
1 Wikipedia page

Citations

dimensions_citation
696 Dimensions

Readers on

mendeley
422 Mendeley
citeulike
2 CiteULike
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1 Connotea
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Title
Genomic instability and myelodysplasia with monosomy 7 consequent to EVI1 activation after gene therapy for chronic granulomatous disease
Published in
Nature Medicine, January 2010
DOI 10.1038/nm.2088
Pubmed ID
Authors

Stefan Stein, Marion G Ott, Stephan Schultze-Strasser, Anna Jauch, Barbara Burwinkel, Andrea Kinner, Manfred Schmidt, Alwin Krämer, Joachim Schwäble, Hanno Glimm, Ulrike Koehl, Carolin Preiss, Claudia Ball, Hans Martin, Gudrun Göhring, Kerstin Schwarzwaelder, Wolf-Karsten Hofmann, Kadin Karakaya, Sandrine Tchatchou, Rongxi Yang, Petra Reinecke, Klaus Kühlcke, Brigitte Schlegelberger, Adrian J Thrasher, Dieter Hoelzer, Reinhard Seger, Christof von Kalle, Manuel Grez

Abstract

Gene-modified autologous hematopoietic stem cells (HSC) can provide ample clinical benefits to subjects suffering from X-linked chronic granulomatous disease (X-CGD), a rare inherited immunodeficiency characterized by recurrent, often life-threatening bacterial and fungal infections. Here we report on the molecular and cellular events observed in two young adults with X-CGD treated by gene therapy in 2004. After the initial resolution of bacterial and fungal infections, both subjects showed silencing of transgene expression due to methylation of the viral promoter, and myelodysplasia with monosomy 7 as a result of insertional activation of ecotropic viral integration site 1 (EVI1). One subject died from overwhelming sepsis 27 months after gene therapy, whereas a second subject underwent an allogeneic HSC transplantation. Our data show that forced overexpression of EVI1 in human cells disrupts normal centrosome duplication, linking EVI1 activation to the development of genomic instability, monosomy 7 and clonal progression toward myelodysplasia.

X Demographics

X Demographics

The data shown below were collected from the profile of 1 X user 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 422 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Netherlands 2 <1%
France 2 <1%
Germany 1 <1%
South Africa 1 <1%
India 1 <1%
Spain 1 <1%
Unknown 414 98%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 86 20%
Researcher 79 19%
Student > Master 55 13%
Student > Bachelor 43 10%
Student > Doctoral Student 23 5%
Other 59 14%
Unknown 77 18%
Readers by discipline Count As %
Agricultural and Biological Sciences 115 27%
Biochemistry, Genetics and Molecular Biology 98 23%
Medicine and Dentistry 60 14%
Immunology and Microbiology 16 4%
Neuroscience 10 2%
Other 42 10%
Unknown 81 19%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 28. 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 17 October 2023.
All research outputs
#1,235,895
of 23,575,346 outputs
Outputs from Nature Medicine
#2,642
of 8,696 outputs
Outputs of similar age
#5,569
of 167,830 outputs
Outputs of similar age from Nature Medicine
#10
of 79 outputs
Altmetric has tracked 23,575,346 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 94th percentile: it's in the top 10% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 8,696 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 100.7. This one has gotten more attention than average, scoring higher than 69% 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 167,830 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 79 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 87% of its contemporaries.