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Mendeley readers
Attention Score in Context
| Title |
Palmitoyl:protein thioesterase (PPT1) inhibitors can act as pharmacological chaperones in infantile Batten disease
|
|---|---|
| Published in |
Biochemical & Biophysical Research Communications, March 2010
|
| DOI | 10.1016/j.bbrc.2010.03.137 |
| Pubmed ID | |
| Authors |
Glyn Dawson, Christina Schroeder, Philip E. Dawson |
| Abstract |
Competitive inhibitors of lysosomal hydrolases (pharmacological chaperones) have been used to treat some lysosomal storage diseases which result from mis-sense mutations and mis-folded protein but have not been tried in Batten disease, for which there is no current therapy. We synthesized a large number of novel, non-hydrolyzable competitive inhibitors of palmitoyl:protein thioesterase (PPT1) and showed that some could act as chemical chaperones. One inhibitor (CS38: betaAGDap(Pal)VKIKK) was taken up by lymphoblasts from patients with mutations leading to the T75P/R151X substitutions and enhanced PPT1 activity 2-fold. A similar 2-fold stimulation with another inhibitor (AcGDap(Palm)GG(R)(7)) was observed in patients with a G108R amino acid substitution in PPT1. Residual PPT1 activity in both was thermally unstable at pH 7.4 (but not at 4.7) consistent with a mis-folded, unstable PPT1 degraded by the ER stress response. Patients with null mutations did not respond to the pharmacological chaperones. |
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 % |
|---|---|---|
| United Kingdom | 1 | 2% |
| Germany | 1 | 2% |
| Unknown | 41 | 95% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Ph. D. Student | 10 | 23% |
| Researcher | 10 | 23% |
| Student > Master | 6 | 14% |
| Professor > Associate Professor | 3 | 7% |
| Student > Bachelor | 2 | 5% |
| Other | 6 | 14% |
| Unknown | 6 | 14% |
| Readers by discipline | Count | As % |
|---|---|---|
| Agricultural and Biological Sciences | 10 | 23% |
| Biochemistry, Genetics and Molecular Biology | 8 | 19% |
| Chemistry | 4 | 9% |
| Medicine and Dentistry | 3 | 7% |
| Neuroscience | 3 | 7% |
| Other | 6 | 14% |
| Unknown | 9 | 21% |
Attention Score in Context
This research output has an Altmetric Attention Score of 12. 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 23 August 2017.
All research outputs
#3,080,964
of 25,374,917 outputs
Outputs from Biochemical & Biophysical Research Communications
#896
of 26,637 outputs
Outputs of similar age
#11,810
of 103,240 outputs
Outputs of similar age from Biochemical & Biophysical Research Communications
#12
of 147 outputs
Altmetric has tracked 25,374,917 research outputs across all sources so far. Compared to these this one has done well and is in the 87th percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 26,637 research outputs from this source. They receive a mean Attention Score of 4.5. This one has done particularly well, scoring higher than 96% 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 103,240 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 88% of its contemporaries.
We're also able to compare this research output to 147 others from the same source and published within six weeks on either side of this one. This one has done particularly well, scoring higher than 91% of its contemporaries.