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Characterization of a DmdEGFP reporter mouse as a tool to investigate dystrophin expression

Overview of attention for article published in Skeletal Muscle, July 2016
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  • Good Attention Score compared to outputs of the same age (66th percentile)

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Title
Characterization of a DmdEGFP reporter mouse as a tool to investigate dystrophin expression
Published in
Skeletal Muscle, July 2016
DOI 10.1186/s13395-016-0095-5
Pubmed ID
Authors

Mina V. Petkova, Susanne Morales-Gonzales, Karima Relizani, Esther Gill, Franziska Seifert, Josefine Radke, Werner Stenzel, Luis Garcia, Helge Amthor, Markus Schuelke

Abstract

Dystrophin is a rod-shaped cytoplasmic protein that provides sarcolemmal stability as a structural link between the cytoskeleton and the extracellular matrix via the dystrophin-associated protein complex (DAPC). Mutations in the dystrophin-encoding DMD gene cause X-linked dystrophinopathies with variable phenotypes, the most severe being Duchenne muscular dystrophy (DMD) characterized by progressive muscle wasting and fibrosis. However, dystrophin deficiency does not only impair the function of skeletal and heart muscle but may also affect other organ systems such as the brain, eye, and gastrointestinal tract. The generation of a dystrophin reporter mouse would facilitate research into dystrophin muscular and extramuscular pathophysiology without the need for immunostaining. We generated a Dmd (EGFP) reporter mouse through the in-frame insertion of the EGFP coding sequence behind the last Dmd exon 79, which is known to be expressed in all major dystrophin isoforms. We analyzed EGFP and dystrophin expression in various tissues and at the single muscle fiber level. Immunostaining of various members of the DAPC was done to confirm the correct subsarcolemmal location of dystrophin-binding partners. We found strong natural EGFP fluorescence at all expected sites of dystrophin expression in the skeletal and smooth muscle, heart, brain, and retina. EGFP fluorescence exactly colocalized with dystrophin immunostaining. In the skeletal muscle, dystrophin and other proteins of the DAPC were expressed at their correct sarcolemmal/subsarcolemmal localization. Skeletal muscle maintained normal tissue architecture, suggesting the correct function of the dystrophin-EGFP fusion protein. EGFP expression could be easily verified in isolated myofibers as well as in satellite cell-derived myotubes. The novel dystrophin reporter mouse provides a valuable tool for direct visualization of dystrophin expression and will allow the study of dystrophin expression in vivo and in vitro in various tissues by live cell imaging.

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X Demographics

The data shown below were collected from the profiles of 6 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 57 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
United Kingdom 1 2%
Japan 1 2%
Unknown 55 96%

Demographic breakdown

Readers by professional status Count As %
Researcher 11 19%
Student > Master 8 14%
Student > Ph. D. Student 7 12%
Lecturer 3 5%
Student > Bachelor 2 4%
Other 10 18%
Unknown 16 28%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 14 25%
Agricultural and Biological Sciences 8 14%
Medicine and Dentistry 5 9%
Veterinary Science and Veterinary Medicine 3 5%
Immunology and Microbiology 2 4%
Other 4 7%
Unknown 21 37%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 4. 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 07 July 2016.
All research outputs
#6,979,392
of 22,880,230 outputs
Outputs from Skeletal Muscle
#210
of 362 outputs
Outputs of similar age
#115,583
of 355,070 outputs
Outputs of similar age from Skeletal Muscle
#7
of 8 outputs
Altmetric has tracked 22,880,230 research outputs across all sources so far. This one has received more attention than most of these and is in the 68th percentile.
So far Altmetric has tracked 362 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 8.2. This one is in the 40th percentile – i.e., 40% of its peers scored the same or lower than it.
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 355,070 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 66% of its contemporaries.
We're also able to compare this research output to 8 others from the same source and published within six weeks on either side of this one.