Figure 2.

Idiopathic pulmonary fibrosis (IPF) biomarkers in the Sftpc mutation model. (A) BAL fluid (BALF) protein content in Sftpc^C121G and Sftpc^WT (n = 15 and 7, respectively). (B) Western blot analysis of BALF for SPP1 (secreted phosphoprotein-1) and MMP7 (matrix metalloproteinase 7) (equal volume loading, 20 μl; n = 5). (C and D) TIMP1 (tissue inhibitor of metalloproteinases-1) and SP-D (surfactant protein D) content in Sftpc^C121G and Sftpc^WT BALF (n = 6). (E) BALF mitochondrial DNA content for MT-ATP6 (mitochondrially encoded ATP synthase membrane subunit 6) and MT-COX1 (mitochondrially encoded cytochrome c oxidase I) (n = 6). (F) Cell count in Sftpc^C121G and Sftpc^WT (n = 24 and 19, respectively). (G) BALF cell differentials as assessed by Giemsa-stained cytospins show increased percentage of granulocytes and reduced percentage of macrophages in Sftpc^C121G. (H–J) CCL2, CCL11, and CCL17 content in Sftpc^C121G and Sftpc^WT BALF (n = 6). Sftpc^C121G and Sftpc^WT compared by Student’s t test, *P < 0.05, **P < 0.01, ***P < 0.001, and ****P < 0.0001.