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Altered Gene Expression in Prefrontal Cortex of a Fabry Disease Mouse Model

Overview of attention for article published in Frontiers in Molecular Neuroscience, June 2018
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Title
Altered Gene Expression in Prefrontal Cortex of a Fabry Disease Mouse Model
Published in
Frontiers in Molecular Neuroscience, June 2018
DOI 10.3389/fnmol.2018.00201
Pubmed ID
Authors

Kai K. Kummer, Theodora Kalpachidou, Miodrag Mitrić, Michiel Langeslag, Michaela Kress

Abstract

Fabry disease is an X-chromosome linked hereditary disease that is caused by loss of function mutations in the α-galactosidase A (α-Gal A) gene, resulting in defective glycolipid degradation and subsequent accumulation of globotriaosylceramide (Gb3) in different tissues, including vascular endothelial cells and neurons in the peripheral and central nervous system. We recently reported a differential gene expression profile of α-Gal A(-/0) mouse dorsal root ganglia, an established animal model of Fabry disease, thereby providing new gene targets that might underlie the neuropathic pain related symptoms. To investigate the cognitive symptoms experienced by Fabry patients, we performed one-color based hybridization microarray expression profiling of prefrontal cortex samples from adult α-Gal A(-/0) mice and age-matched wildtype controls, followed by protein-protein interaction and pathway analyses for the differentially regulated mRNAs. We found that from a total of 381 differentially expressed genes, 135 genes were significantly upregulated, whereas 246 genes were significantly downregulated between α-Gal A(-/0) mice and wildtype controls. Enrichment analysis for downregulated genes revealed mainly immune related pathways, including immune/defense responses, regulation of cytokine production, as well as signaling and transport regulation pathways. Further analysis of the regulated genes revealed a large number of genes involved in neurodegeneration. The current analysis for the first time presents a differential gene expression profile of central nervous system tissue from α-Gal A(-/0) mice, thereby providing novel knowledge on the deregulation and a possible contribution of gene expression to Fabry disease related brain pathologies.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 27 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 27 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 5 19%
Student > Master 4 15%
Student > Bachelor 3 11%
Researcher 3 11%
Professor > Associate Professor 2 7%
Other 3 11%
Unknown 7 26%
Readers by discipline Count As %
Agricultural and Biological Sciences 6 22%
Neuroscience 3 11%
Biochemistry, Genetics and Molecular Biology 2 7%
Pharmacology, Toxicology and Pharmaceutical Science 2 7%
Medicine and Dentistry 2 7%
Other 5 19%
Unknown 7 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 18 July 2018.
All research outputs
#17,981,442
of 23,092,602 outputs
Outputs from Frontiers in Molecular Neuroscience
#2,087
of 2,930 outputs
Outputs of similar age
#237,883
of 328,989 outputs
Outputs of similar age from Frontiers in Molecular Neuroscience
#78
of 118 outputs
Altmetric has tracked 23,092,602 research outputs across all sources so far. This one is in the 19th percentile – i.e., 19% of other outputs scored the same or lower than it.
So far Altmetric has tracked 2,930 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 5.7. This one is in the 21st percentile – i.e., 21% of its peers scored the same or lower than it.
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We're also able to compare this research output to 118 others from the same source and published within six weeks on either side of this one. This one is in the 21st percentile – i.e., 21% of its contemporaries scored the same or lower than it.