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Myotonic Dystrophy—A Progeroid Disease?

Overview of attention for article published in Frontiers in Neurology, July 2018
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Title
Myotonic Dystrophy—A Progeroid Disease?
Published in
Frontiers in Neurology, July 2018
DOI 10.3389/fneur.2018.00601
Pubmed ID
Authors

Peter Meinke, Stefan Hintze, Sarah Limmer, Benedikt Schoser

Abstract

Myotonic dystrophies (DM) are slowly progressing multisystemic disorders caused by repeat expansions in the DMPK or CNBP genes. The multisystemic involvement in DM patients often reflects the appearance of accelerated aging. This is partly due to visible features such as cataracts, muscle weakness, and frontal baldness, but there are also less obvious features like cardiac arrhythmia, diabetes or hypogammaglobulinemia. These aging features suggest the hypothesis that DM could be a segmental progeroid disease. To identify the molecular cause of this characteristic appearance of accelerated aging we compare clinical features of DM to "typical" segmental progeroid disorders caused by mutations in DNA repair or nuclear envelope proteins. Furthermore, we characterize if this premature aging effect is also reflected on the cellular level in DM and investigate overlaps with "classical" progeroid disorders. To investigate the molecular similarities at the cellular level we use primary DM and control cell lines. This analysis reveals many similarities to progeroid syndromes linked to the nuclear envelope. Our comparison on both clinical and molecular levels argues for qualification of DM as a segmental progeroid disorder.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 43 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 43 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 9 21%
Student > Ph. D. Student 6 14%
Student > Master 5 12%
Student > Bachelor 3 7%
Other 2 5%
Other 4 9%
Unknown 14 33%
Readers by discipline Count As %
Medicine and Dentistry 13 30%
Biochemistry, Genetics and Molecular Biology 5 12%
Agricultural and Biological Sciences 4 9%
Neuroscience 2 5%
Social Sciences 1 2%
Other 1 2%
Unknown 17 40%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 3. 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 04 November 2020.
All research outputs
#13,105,954
of 23,098,660 outputs
Outputs from Frontiers in Neurology
#4,940
of 12,015 outputs
Outputs of similar age
#158,990
of 330,303 outputs
Outputs of similar age from Frontiers in Neurology
#116
of 310 outputs
Altmetric has tracked 23,098,660 research outputs across all sources so far. This one is in the 42nd percentile – i.e., 42% of other outputs scored the same or lower than it.
So far Altmetric has tracked 12,015 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.3. This one has gotten more attention than average, scoring higher than 58% 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 330,303 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 50% of its contemporaries.
We're also able to compare this research output to 310 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 62% of its contemporaries.