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Friedreich's ataxia: the vicious circle hypothesis revisited

Overview of attention for article published in BMC Medicine, October 2011
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4 tweeters

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64 Mendeley
Title
Friedreich's ataxia: the vicious circle hypothesis revisited
Published in
BMC Medicine, October 2011
DOI 10.1186/1741-7015-9-112
Pubmed ID
Authors

Aurélien Bayot, Renata Santos, Jean-Michel Camadro, Pierre Rustin

Abstract

Friedreich's ataxia, the most frequent progressive autosomal recessive disorder involving the central and peripheral nervous systems, is mostly associated with unstable expansion of GAA trinucleotide repeats in the first intron of the FXN gene, which encodes the mitochondrial frataxin protein. Since FXN was shown to be involved in Friedreich's ataxia in the late 1990s, the consequence of frataxin loss of function has generated vigorous debate. Very early on we suggested a unifying hypothesis according to which frataxin deficiency leads to a vicious circle of faulty iron handling, impaired iron-sulphur cluster synthesis and increased oxygen radical production. However, data from cell and animal models now indicate that iron accumulation is an inconsistent and late event and that frataxin deficiency does not always impair the activity of iron-sulphur cluster-containing proteins. In contrast, frataxin deficiency appears to be consistently associated with increased sensitivity to reactive oxygen species as opposed to increased oxygen radical production. By compiling the findings of fundamental research and clinical observations we defend here the opinion that the very first consequence of frataxin depletion is indeed an abnormal oxidative status which initiates the pathogenic mechanism underlying Friedreich's ataxia.

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 64 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Spain 2 3%
Portugal 1 2%
Unknown 61 95%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 17 27%
Researcher 13 20%
Student > Postgraduate 5 8%
Student > Bachelor 5 8%
Professor > Associate Professor 5 8%
Other 19 30%
Readers by discipline Count As %
Agricultural and Biological Sciences 28 44%
Biochemistry, Genetics and Molecular Biology 12 19%
Unspecified 6 9%
Medicine and Dentistry 6 9%
Psychology 3 5%
Other 9 14%

Attention Score in Context

This research output has an Altmetric Attention Score of 3. 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 19 October 2011.
All research outputs
#6,398,186
of 12,517,134 outputs
Outputs from BMC Medicine
#1,589
of 2,010 outputs
Outputs of similar age
#47,247
of 95,884 outputs
Outputs of similar age from BMC Medicine
#10
of 15 outputs
Altmetric has tracked 12,517,134 research outputs across all sources so far. This one is in the 48th percentile – i.e., 48% of other outputs scored the same or lower than it.
So far Altmetric has tracked 2,010 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 33.9. This one is in the 20th percentile – i.e., 20% of its peers scored the same or lower than it.
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 95,884 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 50% of its contemporaries.
We're also able to compare this research output to 15 others from the same source and published within six weeks on either side of this one. This one is in the 26th percentile – i.e., 26% of its contemporaries scored the same or lower than it.