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X Demographics
Mendeley readers
Attention Score in Context
| Title |
Skeletal muscle pathology of infantile Pompe disease during long-term enzyme replacement therapy
|
|---|---|
| Published in |
Orphanet Journal of Rare Diseases, June 2013
|
| DOI | 10.1186/1750-1172-8-90 |
| Pubmed ID | |
| Authors |
Sean N Prater, Trusha T Patel, Anne F Buckley, Hanna Mandel, Eugene Vlodavski, Suhrad G Banugaria, Erin J Feeney, Nina Raben, Priya S Kishnani |
| Abstract |
Pompe disease is an autosomal recessive metabolic neuromuscular disorder caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). It has long been believed that the underlying pathology leading to tissue damage is caused by the enlargement and rupture of glycogen-filled lysosomes. Recent studies have also implicated autophagy, an intracellular lysosome-dependent degradation system, in the disease pathogenesis. In this study, we characterize the long-term impact of enzyme replacement therapy (ERT) with recombinant human GAA (rhGAA) on lysosomal glycogen accumulation and autophagy in some of the oldest survivors with classic infantile Pompe disease (IPD). |
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.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Mexico | 1 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 1 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 74 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United States | 1 | 1% |
| Philippines | 1 | 1% |
| Italy | 1 | 1% |
| Unknown | 71 | 96% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Researcher | 12 | 16% |
| Student > Ph. D. Student | 10 | 14% |
| Other | 9 | 12% |
| Student > Doctoral Student | 7 | 9% |
| Student > Bachelor | 7 | 9% |
| Other | 9 | 12% |
| Unknown | 20 | 27% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 22 | 30% |
| Biochemistry, Genetics and Molecular Biology | 11 | 15% |
| Agricultural and Biological Sciences | 10 | 14% |
| Neuroscience | 2 | 3% |
| Engineering | 2 | 3% |
| Other | 5 | 7% |
| Unknown | 22 | 30% |
Attention Score in Context
This research output has an Altmetric Attention Score of 10. 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 28 October 2018.
All research outputs
#3,105,194
of 22,712,476 outputs
Outputs from Orphanet Journal of Rare Diseases
#414
of 2,602 outputs
Outputs of similar age
#27,850
of 196,704 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#4
of 29 outputs
Altmetric has tracked 22,712,476 research outputs across all sources so far. Compared to these this one has done well and is in the 86th percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 2,602 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.5. This one has done well, scoring higher than 83% 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 196,704 tracked outputs that were published within six weeks on either side of this one in any source. This one has done well, scoring higher than 85% of its contemporaries.
We're also able to compare this research output to 29 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 86% of its contemporaries.