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Figure 1.

Clinical features of PKS.

(A)–(D). Full face and profile views of the facial features in two unrelated children with PKS. (E)–(G). Swirly hypo- and hyperpigmentation of the skin in 3 children with PKS. (H)–(J). Broad first toes in 3 children with PKS.

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Table 1.

Sample/mosaic ratio information.

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Figure 2.

Patient and control sample clustering.

Red squares/circles represent PKS patients and Green squares/circles represent control samples. (A) Unsupervised clustering of 26 samples using all genes. (B) Unsupervised clustering of 26 samples using genes located on 12p.(C) PCA result. Proportion of Variance % (PC1-24.818, p 0.001; PC2-17.814, p 0.022; PC3-0.022, p 0.772).

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Figure 3.

Gene expression levels in PKS.

a: Mosaic 12p expression level correlation: Average expression of 171 genes on 12p in patients and controls. Red bars are patients and Grey is controls. X-axis represents samples and Y-axis shows average expression of genes on 12p. b: Level of i12p mosaicism compared to 12p gene expression: average of 64 genes with expression higher than the 3rd quartile of global expression distribution, were correlated to mosaicism%. The blue line in the figure is the fitting line of linear regression. Red diamonds are the probands and green are the controls.

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Table 2.

List of upregulated genes on 12p in PKS probands.

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Table 2 Expand

Figure 4.

BOX plot of HOX gene clusters.

HOX A is down- and HOX B Cluster is up-regulated in PKS patients.

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Figure 5.

Ingenuity pathway analysis of dysregulated genes in PKS.

Green circles represent the genes down-regulated in PKS probands, and red circles represent the genes up-regulated in PKS probands.

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Figure 6.

Schematic illustration of disease mechanism of PKS.

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