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Overexpression of Cystathionine γ-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3

Overview of attention for article published in Molecular Medicine, October 2015
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Title
Overexpression of Cystathionine γ-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3
Published in
Molecular Medicine, October 2015
DOI 10.2119/molmed.2015.00221
Pubmed ID
Authors

Pauline M. Snijder, Madina Baratashvili, Nicola A. Grzeschik, Henri G. D. Leuvenink, Lucas Kuijpers, Sippie Huitema, Onno Schaap, Ben N. G. Giepmans, Jeroen Kuipers, Jan Lj Miljkovic, Aleksandra Mitrovic, Eelke M. Bos, Csaba Szabó, Harm H. Kampinga, Pascale F. Dijkers, Wilfred F. A. den Dunnen, Milos R. Filipovic, Harry van Goor, Ody C. M. Sibon

Abstract

Spinocerebellar ataxia type 3 (SCA3) is a polyglutamine (polyQ) disorder caused by a CAG repeat expansion in the ATXN3 gene resulting in toxic protein aggregation. Inflammation and oxidative stress are considered secondary factors contributing to the progression of this neurodegenerative disease. There is no cure that halts or reverses the progressive neurodegeneration of SCA3. Here we show that overexpression of cystathionine γ-lyase, a central enzyme in cysteine metabolism, is protective in a Drosophila model for SCA3. SCA3 flies show eye degeneration, increased oxidative stress, insoluble protein aggregates, reduced levels of protein persulfidation and increased activation of the innate immune response. Overexpression of Drosophila cystathionine γ-lyase restores protein persulfidation, decreases oxidative stress, dampens the immune response and improves SCA3-associated tissue degeneration. Levels of insoluble protein aggregates are not altered, therefore the data implicate a modifying role of cystathionine γ-lyase in ameliorating the downstream consequence of protein aggregation leading to protection against SCA3-induced tissue degeneration. The cystathionine γ-lyase expression is decreased in affected brain tissue of SCA3 patients, suggesting that enhancers of cystathionine γ-lyase expression or activity are attractive candidates for future therapies.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 46 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 46 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 9 20%
Student > Ph. D. Student 8 17%
Student > Master 7 15%
Professor 5 11%
Student > Bachelor 4 9%
Other 4 9%
Unknown 9 20%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 16 35%
Agricultural and Biological Sciences 7 15%
Chemistry 4 9%
Medicine and Dentistry 3 7%
Neuroscience 3 7%
Other 1 2%
Unknown 12 26%
Attention Score in Context

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 16 October 2015.
All research outputs
#17,284,057
of 25,382,035 outputs
Outputs from Molecular Medicine
#884
of 1,266 outputs
Outputs of similar age
#173,068
of 289,561 outputs
Outputs of similar age from Molecular Medicine
#10
of 17 outputs
Altmetric has tracked 25,382,035 research outputs across all sources so far. This one is in the 21st percentile – i.e., 21% of other outputs scored the same or lower than it.
So far Altmetric has tracked 1,266 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 8.2. This one is in the 21st percentile – i.e., 21% 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 289,561 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 31st percentile – i.e., 31% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 17 others from the same source and published within six weeks on either side of this one. This one is in the 47th percentile – i.e., 47% of its contemporaries scored the same or lower than it.