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Purkinje cell injury, structural plasticity and fusion in patients with Friedreich’s ataxia

Overview of attention for article published in Acta Neuropathologica Communications, May 2016
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Title
Purkinje cell injury, structural plasticity and fusion in patients with Friedreich’s ataxia
Published in
Acta Neuropathologica Communications, May 2016
DOI 10.1186/s40478-016-0326-3
Pubmed ID
Authors

Kevin C. Kemp, Amelia J. Cook, Juliana Redondo, Kathreena M. Kurian, Neil J. Scolding, Alastair Wilkins

Abstract

Purkinje cell pathology is a common finding in a range of inherited and acquired cerebellar disorders, with the degree of Purkinje cell injury dependent on the underlying aetiology. Purkinje cells have an unparalleled resistance to insult and display unique regenerative capabilities within the central nervous system. Their response to cell injury is not typical of most neurons and likely represents both degenerative, compensatory and regenerative mechanisms. Here we present a pathological study showing novel and fundamental insights into Purkinje cell injury, remodelling and repair in Friedreich's ataxia; the most common inherited ataxia. Analysing post-mortem cerebellum tissue from patients who had Friedreich's ataxia, we provide evidence of significant injury to the Purkinje cell axonal compartment with relative preservation of both the perikaryon and its extensive dendritic arborisation. Axonal remodelling of Purkinje cells was clearly elevated in the disease. For the first time in a genetic condition, we have also shown a disease-related increase in the frequency of Purkinje cell fusion and heterokaryon formation in Friedreich's ataxia cases; with evidence that underlying levels of cerebellar inflammation influence heterokaryon formation. Our results together further demonstrate the Purkinje cell's unique plasticity and regenerative potential. Elucidating the biological mechanisms behind these phenomena could have significant clinical implications for manipulating neuronal repair in response to neurological injury.

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The data shown below were collected from the profiles of 2 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 58 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 58 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 12 21%
Researcher 11 19%
Student > Bachelor 6 10%
Student > Master 5 9%
Professor > Associate Professor 5 9%
Other 10 17%
Unknown 9 16%
Readers by discipline Count As %
Neuroscience 15 26%
Medicine and Dentistry 9 16%
Agricultural and Biological Sciences 9 16%
Biochemistry, Genetics and Molecular Biology 6 10%
Pharmacology, Toxicology and Pharmaceutical Science 2 3%
Other 5 9%
Unknown 12 21%

Attention Score in Context

This research output has an Altmetric Attention Score of 1. 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 25 May 2016.
All research outputs
#5,869,513
of 7,757,128 outputs
Outputs from Acta Neuropathologica Communications
#289
of 363 outputs
Outputs of similar age
#187,740
of 268,889 outputs
Outputs of similar age from Acta Neuropathologica Communications
#27
of 31 outputs
Altmetric has tracked 7,757,128 research outputs across all sources so far. This one is in the 13th percentile – i.e., 13% of other outputs scored the same or lower than it.
So far Altmetric has tracked 363 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.1. This one is in the 7th percentile – i.e., 7% 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 268,889 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 16th percentile – i.e., 16% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 31 others from the same source and published within six weeks on either side of this one. This one is in the 9th percentile – i.e., 9% of its contemporaries scored the same or lower than it.