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Figure 1 | Orphanet Journal of Rare Diseases

Figure 1

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

Figure 1

Immune activity is hyperactive in CHS skin fibroblasts at baseline. A. The differences in the expression of genes encoding 84 inflammation-related factors between CHS cells and control cells (Ctrl) at baseline are shown volcano plot. 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). Three genes, TLR-1, IL-1R1 and CD14 (listed in Table 3) are significantly (p < 0.05) down-regulated with 2 to 3 fold changes. RT-qPCR data reveals that compared to control cells at baseline: B. Expression of genes encoding TLR-2 and −4 are down-regulated in CHS cells at baseline; C. TLR-3 gene expression is enhanced in CHS cells at baseline. D. Expression of genes encoding IL1β and IL-6 are up-regulated in CHS cells at baseline; E. COX-2 gene expression is up-regulated in CHS cells at baseline. Experiments were done twice, in triplicate, with comparable results. Statistically differences were accessed by a student’s t test (*p < 0.05 and **p < 0.01).

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