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. 2019 Jul 18;54(1):1802041. doi: 10.1183/13993003.02041-2018

FIGURE 3.

FIGURE 3

Effect of S100A3 and S100A13 mutations on protein expression. AU: arbitrary units; IPF: idiopathic pulmonary fibrosis; GAPDH: glyceraldehyde 3-phosphate dehydrogenase. a) Upper images show immunofluorescence micrographs demonstrating reduced expression of S100A3 and S100A13 proteins in lung tissue from a normal control and an affected family member (F1:IV-2). Scale bar: 10 µm. Magnified areas of the indicated portions are shown in the lower images. b) Relative protein expression of S100A3 and S100A13 in normal control and lung tissues from two independent patients shown together with relative protein expression in an IPF patient. Histograms are mean±sd intensity of multiple fields in the stained samples. c) Confocal fluorescence laser scanning micrographs showing the reduced expression of S100A3 and S100A13 proteins in skin fibroblasts isolated from patients compared with controls and the corresponding Western blots. Data are representative of three independent experiments with cells isolated from two patients and two controls. Scale bar: 20 µm. d) Relative expression of S100A3 and S100A13 mRNA in skin fibroblasts isolated from normal controls and patients. Data are representative of at least three independent experiments. p-values are indicated when appropriate.