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Riluzole partially restores RNA polymerase III complex assembly in cells expressing the leukodystrophy-causative variant POLR3B R103H

Overview of attention for article published in Molecular Brain, November 2022
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  • In the top 5% of all research outputs scored by Altmetric
  • Among the highest-scoring outputs from this source (#25 of 1,198)
  • High Attention Score compared to outputs of the same age (94th percentile)
  • High Attention Score compared to outputs of the same age and source (99th percentile)

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4 news outlets
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10 X users

Citations

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5 Dimensions

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7 Mendeley
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Title
Riluzole partially restores RNA polymerase III complex assembly in cells expressing the leukodystrophy-causative variant POLR3B R103H
Published in
Molecular Brain, November 2022
DOI 10.1186/s13041-022-00974-z
Pubmed ID
Authors

Maxime Pinard, Samaneh Dastpeyman, Christian Poitras, Geneviève Bernard, Marie-Soleil Gauthier, Benoit Coulombe

Abstract

The mechanism of assembly of RNA polymerase III (Pol III), the 17-subunit enzyme that synthesizes tRNAs, 5 S rRNA, and other small-nuclear (sn) RNAs in eukaryotes, is not clearly understood. The recent discovery of the HSP90 co-chaperone PAQosome (Particle for Arrangement of Quaternary structure) revealed a function for this machinery in the biogenesis of nuclear RNA polymerases. However, the connection between Pol III subunits and the PAQosome during the assembly process remains unexplored. Here, we report the development of a mass spectrometry-based assay that allows the characterization of Pol III assembly. This assay was used to dissect the stages of Pol III assembly, to start defining the function of the PAQosome in this process, to dissect the assembly defects driven by the leukodystrophy-causative R103H substitution in POLR3B, and to discover that riluzole, an FDA-approved drug for alleviation of ALS symptoms, partly corrects these assembly defects. Together, these results shed new light on the mechanism and regulation of human nuclear Pol III biogenesis.

X Demographics

X Demographics

The data shown below were collected from the profiles of 10 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 7 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 7 100%

Demographic breakdown

Readers by professional status Count As %
Professor > Associate Professor 2 29%
Student > Bachelor 1 14%
Researcher 1 14%
Unknown 3 43%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 2 29%
Nursing and Health Professions 1 14%
Chemistry 1 14%
Unknown 3 43%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 34. 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 December 2022.
All research outputs
#1,180,132
of 25,368,786 outputs
Outputs from Molecular Brain
#25
of 1,198 outputs
Outputs of similar age
#26,121
of 486,381 outputs
Outputs of similar age from Molecular Brain
#1
of 25 outputs
Altmetric has tracked 25,368,786 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 95th percentile: it's in the top 5% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 1,198 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 6.9. This one has done particularly well, scoring higher than 97% 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 486,381 tracked outputs that were published within six weeks on either side of this one in any source. This one has done particularly well, scoring higher than 94% of its contemporaries.
We're also able to compare this research output to 25 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 99% of its contemporaries.