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Linkage of a prion protein missense variant to Gerstmann–Sträussler syndrome

Overview of attention for article published in Nature, March 1989
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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 (91st percentile)

Mentioned by

blogs
1 blog
policy
2 policy sources
patent
18 patents

Citations

dimensions_citation
685 Dimensions

Readers on

mendeley
132 Mendeley
citeulike
1 CiteULike
Title
Linkage of a prion protein missense variant to Gerstmann–Sträussler syndrome
Published in
Nature, March 1989
DOI 10.1038/338342a0
Pubmed ID
Authors

Karen Hsiao, Harry F. Baker, Tim J. Crow, Mark Poulter, Frank Owen, Joseph D. Terwilliger, David Westaway, Jurg Ott, Stanley B. Prusiner

Abstract

Gerstmann-Sträussler syndrome is a rare familial neurodegenerative condition that is vertically transmitted, in an apparently autosomal dominant way. It can also be horizontally transmitted to non-human primates and rodents through intracerebral inoculation of brain homogenates from patients with the disease. The exact incidence of the syndrome is unknown but is estimated to be between one and ten per hundred million. Patients initially suffer from ataxia or dementia and deteriorate until they die, in one to ten years. Protease-resistant prion protein (PrP) and PrP-immunoreactive amyloid plaques with characteristic morphology accumulate in the brains of these patients. Current diagnostic criteria for Gerstmann-Sträussler syndrome incorporate clinical and neuropathological features, as animal transmission studies can be unreliable. PrP is implicated in the pathogenesis and transmission of the condition and in scrapie, an equivalent animal disease. It was discovered by enriching scrapie-infected hamster brain fractions for infectivity. Because there is compelling evidence that the scrapie isoform of PrP is a necessary component of the infectious particle, it seemed possible that the PrP gene on the short arm of human chromosome 20 in Gerstmann-Sträussler syndrome might be abnormal. We show here that PrP codon 102 is linked to the putative gene for the syndrome in two pedigrees, providing the best evidence to date that this familial condition is inherited despite also being infectious, and that substitution of leucine for proline at PrP codon 102 may lead to the development of Gerstmann-Sträussler syndrome.

Mendeley readers

The data shown below were compiled from readership statistics for 132 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
United States 3 2%
United Kingdom 1 <1%
Lithuania 1 <1%
Netherlands 1 <1%
Unknown 126 95%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 28 21%
Researcher 26 20%
Student > Bachelor 21 16%
Student > Master 16 12%
Professor 10 8%
Other 24 18%
Unknown 7 5%
Readers by discipline Count As %
Agricultural and Biological Sciences 42 32%
Biochemistry, Genetics and Molecular Biology 26 20%
Neuroscience 19 14%
Medicine and Dentistry 16 12%
Chemistry 9 7%
Other 9 7%
Unknown 11 8%

Attention Score in Context

This research output has an Altmetric Attention Score of 17. 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 15 July 2018.
All research outputs
#876,797
of 13,226,211 outputs
Outputs from Nature
#27,087
of 68,925 outputs
Outputs of similar age
#20,197
of 233,907 outputs
Outputs of similar age from Nature
#765
of 995 outputs
Altmetric has tracked 13,226,211 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 93rd percentile: it's in the top 10% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 68,925 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 75.0. This one has gotten more attention than average, scoring higher than 60% 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 233,907 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 91% of its contemporaries.
We're also able to compare this research output to 995 others from the same source and published within six weeks on either side of this one. This one is in the 23rd percentile – i.e., 23% of its contemporaries scored the same or lower than it.