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

Frontiers in Molecular Neuroscience
Kai K KummerMichaela 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, includ...Continue Reading

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Citations

Aug 26, 2020·Neurochemistry International·Libia Catalina Salinas CastellanosCarina Weissmann
Dec 2, 2020·Pain·Anthony J Burand, Cheryl L Stucky
Jul 22, 2021·Molecular Pain·Carina WeissmannOsvaldo D Uchitel

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Datasets Mentioned

BETA
GSE110645

Methods Mentioned

BETA
transgenic
feature extraction
PCR

Software Mentioned

Normfinder
R ggplot
R Development Core Team
Bestkeeper
GeneSpring GX
geNorm
R

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