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Fig 1.

GalCer induces sex-specific alterations in gene expression in Cln3Δex7/8 mouse brain.

Bar chart indicating differences in numbers of significant differentially expressed genes affected by GalCer compared to vehicle treatment in Cln3Δex7/8 male and female mice. A cut-off p-value < 0.05 and fold-change ≥ ±1.3 were assumed to identify genes significantly modulated by GalCer treatment.

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Fig 2.

Differentially expressed genes in GalCer versus vehicle-treated male and female Cln3Δex7/8 mouse brain.

2-dimentional heat maps of (a) 66 DEGs in male Cln3Δex7/8 mice, and (b) 30 DEGs in female Cln3Δex7/8 mice. Heat maps show mRNA abundance intensities of differentially expressed genes in profiled samples. Robust Multi-Array Average (RMA) preprocessed data is transformed to z-scores. The legend represents relative over- (red) and under-expression (green). Labeling at the top represents Vehicle control samples and GalCer-treated samples. A cut-off p-value < 0.05 and fold-change ≥ ±1.3 were assumed to identify genes significantly modulated by GalCer treatment.

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Fig 3.

Bar chart of GO enrichment scores for DEGs according to biological processes in male/female Cln3Δex7/8 mouse brain.

Gene expression in GalCer- versus vehicle-treated male (a) and female (b) Cln3Δex7/8 mouse brain. Differential expression is assumed with a p-value < 0.05, and a gene count > 2 of the DEGs. Functional categories of DEGs were obtained using GO annotations from the Partek Genomics Suite classification system.

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Fig 4.

Forest plot of GO for DEGs in biological processes in male Cln3Δex7/8 mouse brain.

Gene expression in GalCer- versus vehicle-treated Cln3Δex7/8 male mouse brain. The criterion for differential expression assumes a p-value < 0.05. Forest plots of biological processes including (a) developmental, (b) cellular, and (c) behavioral processes depict percentage of down- (green) or up-regulated (red) genes in each category.

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Fig 5.

Forest plot of GO for DEGs in biological processes in female Cln3Δex7/8 mouse brain.

Gene expression in GalCer versus vehicle-treated female Cln3Δex7/8 mouse brain. The criterion for differential expression assumes a p-value < 0.05. Forest plots of biological processes of (a) developmental, (b) cellular, and (c) behavioral processes depict percentage of down- (green) or up-regulated (red) genes in each category.

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Fig 6.

Gene-disease relationship network of significant DEGs in GalCer- versus vehicle-treated male Cln3Δex7/8 mice.

Pathway Studio software generated network interactions between diseases and most significant DEGs in male Cln3Δex7/8 mouse brain (p-value < 0.05, stringency ≥ ± 1.3 fold-change in expression). Depiction of genes is by their gene symbols. Upregulated genes are highlighted with a red halo and downregulated genes are highlighted with a green halo. Indirect regulation is indicated by dotted gray lines.

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Fig 7.

Gene-disease relationship network of significant DEGs in GalCer- versus vehicle-treated female Cln3Δex7/8 mice.

Pathway Studio software generated network interactions between diseases and most significant DEGs in female Cln3Δex7/8 mouse brain (p-value < 0.05, stringency ≥ ± 1.3 fold-change in expression). Depiction of genes is by gene symbols. Upregulated genes are highlighted with a red halo and downregulated genes are highlighted with a green halo. Indirect regulation is indicated by dotted gray lines.

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Fig 8.

Validation of microarray analysis results with qRT-PCR in male and female Cln3Δex7/8 mouse brain.

Relative mRNA expression measured by qRT-PCR. (a) The following differentially expressed genes in male Cln3Δex7/8 mouse brains were randomly chosen: Atp6v0a1, Braf, Dnm3, Prkacb, Gabbr, Grm1, and C1qb. Values are means of the fold changes normalized to Gapdh mRNA expression, with standard errors represented by vertical bars.*p-value < 0.05 and ** p-value < 0.01 by Student’s t-test (n = 4). (b) The following differentially expressed genes in female Cln3Δex7/8 mouse brains were randomly chosen: Fos, Prlr, and Fdft1. Values are means of the fold changes normalized to Gapdh mRNA expression, with standard errors represented by vertical bars. *** p-value < 0.001 and **** p-value < 0.0001 by Student’s t-test (n = 4).

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Fig 9.

Comparative analysis of DEGs in male and female Cln3Δex7/8 mouse brain.

Depiction of DEGs selected in male and female Cln3Δex7/8 mouse brain based on gene expression in GalCer compared to vehicle-treated Cln3Δex7/8 mice of both genders. The criterion for differential expression was a p-value < 0.05, and a stringency ≥ ± 1.3-fold change in expression. (a) A total number of 96 genes, with 65 and 29 genes exclusive for male and female Cln3Δex7/8 mouse brain, respectively, shown. One gene appears in the overlapping region, common to both datasets. (b) Fold change and p-value of the gene (Ddit4) common to male and female datasets. (c) Schematic representation of the effect of GalCer on the function of Ddit4 in brain.

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