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Figure 2 | Orphanet Journal of Rare Diseases

Figure 2

From: Skin fibroblasts from individuals with Chediak-Higashi Syndrome (CHS) exhibit hyposensitive immunogenic response

Figure 2

Skin fibroblasts from individuals with CHS exhibit a hyposensitive immune response when challenged with LPS. The differences in the expression of genes encoding 84 inflammation-related factors are shown via volcano plots (A-C). Y-axis shows the values of minus Log10 (p-value) and X-axis shows the values of Log2 (Fold change of CHS vs. control). Red circles fall to the right of the red line represent genes increases more than 2-fold and green circles fall to the left of the green line represents genes decreases more than 2 fold. Circles above the blue line represent genes with changes of statistically significantly differences (p < 0.05 by t-test). A. Expression of genes encoding inflammation-related factors are repressed significantly in LPS-treated CHS cells compared to LPS-treated control (Ctrl) cells (shown in Table 5). B. Only 8 genes (shown in Table 6) are up-regulated significantly in LPS treated CHS cells compared to untreated CHS cells. C. 28 genes (shown in Table 7) are up-regulated significantly in LPS-treated control cells compared to untreated control cells. D. RT-qPCR data reveals that when normalized to their respective untreated cells, LPS-treated CHS cells exhibit significantly lower expression of genes encoding IL-6, IL-1β and COX-2 compared to LPS-treated control cells. E. IL-6 expression in cell culture media evaluated by ELISA shows that IL-6 is significantly lower in culture media of CHS cells treated with or without LPS. Experiments were done twice, in triplicate, with comparable results. Statistically differences were accessed by a student’s t test in panel D (**p < 0.01 and ***p < 0.001) and by one-way ANOVA with Tukey’s post hoc test in panel E (*p < 0.05).

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