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Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein

Overview of attention for article published in Acta Neuropathologica Communications, February 2018
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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 (#38 of 1,394)
  • High Attention Score compared to outputs of the same age (95th percentile)
  • High Attention Score compared to outputs of the same age and source (81st percentile)

Mentioned by

news
6 news outlets
blogs
2 blogs
twitter
5 tweeters

Citations

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18 Dimensions

Readers on

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29 Mendeley
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Title
Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein
Published in
Acta Neuropathologica Communications, February 2018
DOI 10.1186/s40478-018-0516-2
Pubmed ID
Authors

Brent Race, Katie Williams, Andrew G. Hughson, Casper Jansen, Piero Parchi, Annemieke J. M. Rozemuller, Bruce Chesebro

Abstract

Human familial prion diseases are associated with mutations at 34 different prion protein (PrP) amino acid residues. However, it is unclear whether infectious prions are found in all cases. Mutant PrP itself may be neurotoxic, or alternatively, PrP mutation might predispose to spontaneous formation of infectious PrP isoforms. Previous reports demonstrated transmission to animal models by human brain tissue expressing 7 different PrP mutations, but 3 other mutations were not transmissible. In the present work, we tested transmission using brain homogenates from patients expressing 3 untested PrP mutants: G131V, Y226X, and Q227X. Human brain homogenates were injected intracerebrally into tg66 transgenic mice overexpressing human PrP. Mice were followed for nearly 800 days.From 593 to 762 dpi, 4 of 8 mice injected with Y226X brain had PrPSc detectable in brain by immunostaining, immunoblot, and PrP amyloid seeding activity assayed by RT-QuIC. From 531 to 784 dpi, 11 of 11 G131V-injected mice had PrPSc deposition in brain, but none were positive by immunoblot or RT-QuIC assay. In contrast, from 529 to 798 dpi, no tg66 mice injected with Q227X brain had PrPSc or PrP amyloid seeding activity detectable by these methods. Y226X is the only one of 4 known PrP truncations associated with familial disease which has been shown to be transmissible. This transmission of prion infectivity from a patient expressing truncated human PrP may have implications for the spread and possible transmission of other aggregated truncated proteins in prion-like diseases such as Alzheimer's disease, Parkinson's disease and tauopathies.

Twitter Demographics

The data shown below were collected from the profiles of 5 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 29 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 29 100%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 5 17%
Researcher 5 17%
Other 4 14%
Student > Master 3 10%
Student > Ph. D. Student 1 3%
Other 2 7%
Unknown 9 31%
Readers by discipline Count As %
Neuroscience 5 17%
Biochemistry, Genetics and Molecular Biology 3 10%
Medicine and Dentistry 3 10%
Immunology and Microbiology 3 10%
Engineering 2 7%
Other 4 14%
Unknown 9 31%

Attention Score in Context

This research output has an Altmetric Attention Score of 60. 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 09 April 2018.
All research outputs
#611,985
of 23,025,074 outputs
Outputs from Acta Neuropathologica Communications
#38
of 1,394 outputs
Outputs of similar age
#15,636
of 331,055 outputs
Outputs of similar age from Acta Neuropathologica Communications
#4
of 22 outputs
Altmetric has tracked 23,025,074 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 97th percentile: it's in the top 5% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 1,394 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 12.8. 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 331,055 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 95% of its contemporaries.
We're also able to compare this research output to 22 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 81% of its contemporaries.