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Short peptides from leucyl-tRNA synthetase rescue disease-causing mitochondrial tRNA point mutations

Overview of attention for article published in Human Molecular Genetics, December 2015
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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 (87th percentile)
  • Above-average Attention Score compared to outputs of the same age and source (61st percentile)

Mentioned by

news
1 news outlet
twitter
4 tweeters

Citations

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14 Dimensions

Readers on

mendeley
25 Mendeley
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Title
Short peptides from leucyl-tRNA synthetase rescue disease-causing mitochondrial tRNA point mutations
Published in
Human Molecular Genetics, December 2015
DOI 10.1093/hmg/ddv619
Pubmed ID
Authors

Elena Perli, Annarita Fiorillo, Carla Giordano, Annalinda Pisano, Arianna Montanari, Paola Grazioli, Antonio F. Campese, Patrizio Di Micco, Helen A. Tuppen, Ilaria Genovese, Elena Poser, Carmela Preziuso, Robert W. Taylor, Veronica Morea, Gianni Colotti, Giulia d'Amati

Abstract

Mutations in mitochondrial (mt) genes coding for mt-tRNAs are responsible for a range of syndromes, for which no effective treatment is available. We recently showed that the carboxy-terminal domain of human mt-leucyl tRNA synthetase (Cterm) rescues the pathologic phenotype associated either with the m.3243A>G mutation in mt-tRNA(Leu(UUR)) or with mutations in the mt-tRNA(Ile), both of which are aminoacylated by Class I mt-aminoacyl-tRNA synthetases (mt-aaRSs).Here we show, by using the human transmitochondrial cybrid model, that the Cterm is also able to improve the phenotype caused by the m.8344A>G mutation in mt-tRNA(Lys), aminoacylated by a Class II aaRS. Importantly, we demonstrate that the same rescuing ability is retained by two Cterm-derived short peptides, β30_31 and β32_33, which are effective towards both the m.8344A>G and the m.3243A>G mutations. Furthermore, we provide in vitro evidence that these peptides bind with high affinity wild-type and mutant human mt-tRNA(Leu(UUR)) and mt-tRNA(Lys), and stabilize mutant mt-tRNA(Leu(UUR)). In conclusion, we demonstrate that small Cterm-derived peptides can be effective tools to rescue cellular defects caused by mutations in a wide range of mt-tRNAs.

Twitter Demographics

The data shown below were collected from the profiles of 4 tweeters who shared this research output. Click here to find out more about how the information was compiled.

Mendeley readers

The data shown below were compiled from readership statistics for 25 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 25 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 6 24%
Student > Master 3 12%
Student > Postgraduate 2 8%
Student > Ph. D. Student 2 8%
Student > Bachelor 1 4%
Other 2 8%
Unknown 9 36%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 7 28%
Agricultural and Biological Sciences 3 12%
Unspecified 2 8%
Medicine and Dentistry 2 8%
Psychology 1 4%
Other 1 4%
Unknown 9 36%

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 01 March 2016.
All research outputs
#1,113,805
of 12,025,923 outputs
Outputs from Human Molecular Genetics
#545
of 6,204 outputs
Outputs of similar age
#40,569
of 333,859 outputs
Outputs of similar age from Human Molecular Genetics
#49
of 128 outputs
Altmetric has tracked 12,025,923 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 90th percentile: it's in the top 10% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 6,204 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 6.0. This one has done particularly well, scoring higher than 91% 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 333,859 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 87% of its contemporaries.
We're also able to compare this research output to 128 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 61% of its contemporaries.