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Co-expression of C9orf72 related dipeptide-repeats over 1000 repeat units reveals age- and combination-specific phenotypic profiles in Drosophila

Overview of attention for article published in Acta Neuropathologica Communications, September 2020
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About this Attention Score

  • In the top 5% of all research outputs scored by Altmetric
  • Among the highest-scoring outputs from this source (#25 of 958)
  • High Attention Score compared to outputs of the same age (93rd percentile)

Mentioned by

news
2 news outlets
blogs
1 blog
twitter
26 tweeters

Readers on

mendeley
13 Mendeley
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Title
Co-expression of C9orf72 related dipeptide-repeats over 1000 repeat units reveals age- and combination-specific phenotypic profiles in Drosophila
Published in
Acta Neuropathologica Communications, September 2020
DOI 10.1186/s40478-020-01028-y
Pubmed ID
Authors

Ryan J. H. West, Joanne L. Sharpe, André Voelzmann, Anna L. Munro, Ines Hahn, Richard A. Baines, Stuart Pickering-Brown

Abstract

A large intronic hexanucleotide repeat expansion (GGGGCC) within the C9orf72 (C9orf72-SMCR8 Complex Subunit) locus is the most prevalent genetic cause of both Frontotemporal Dementia (FTD) and Motor Neuron Disease (MND). In patients this expansion is typically hundreds to thousands of repeat units in length. Repeat associated non-AUG translation of the expansion leads to the formation of toxic, pathological Dipeptide-Repeat Proteins (DPRs). To date there remains a lack of in vivo models expressing C9orf72 related DPRs with a repeat length of more than a few hundred repeats. As such our understanding of how physiologically relevant repeat length DPRs effect the nervous system in an ageing in vivo system remains limited. In this study we generated Drosophila models expressing DPRs over 1000 repeat units in length, a known pathological length in humans. Using these models, we demonstrate each DPR exhibits a unique, age-dependent, phenotypic and pathological profile. Furthermore, we show co-expression of specific DPR combinations leads to distinct, age-dependent, phenotypes not observed through expression of single DPRs. We propose these models represent a unique, in vivo, tool for dissecting the molecular mechanisms implicated in disease pathology, opening up new avenues in the study of both MND and FTD.

Twitter Demographics

The data shown below were collected from the profiles of 26 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 13 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 13 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 5 38%
Student > Bachelor 2 15%
Student > Master 2 15%
Professor 1 8%
Other 1 8%
Other 1 8%
Unknown 1 8%
Readers by discipline Count As %
Neuroscience 5 38%
Biochemistry, Genetics and Molecular Biology 3 23%
Agricultural and Biological Sciences 2 15%
Philosophy 1 8%
Unknown 2 15%

Attention Score in Context

This research output has an Altmetric Attention Score of 41. 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 06 October 2020.
All research outputs
#559,664
of 16,157,205 outputs
Outputs from Acta Neuropathologica Communications
#25
of 958 outputs
Outputs of similar age
#19,261
of 304,470 outputs
Outputs of similar age from Acta Neuropathologica Communications
#1
of 1 outputs
Altmetric has tracked 16,157,205 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 96th percentile: it's in the top 5% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 958 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 10.7. 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 304,470 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 93% of its contemporaries.
We're also able to compare this research output to 1 others from the same source and published within six weeks on either side of this one. This one has scored higher than all of them