Expression of fibroblast growth factor 9 in normal human lung and idiopathic pulmonary fibrosis

TitleExpression of fibroblast growth factor 9 in normal human lung and idiopathic pulmonary fibrosis
Publication TypeJournal Article
Year of Publication2013
AuthorsCoffey E, Newman DR, Sannes PL
JournalJ Histochem Cytochem
Date Published2013 Sep
KeywordsAdult, Cells, Cultured, Fibroblast Growth Factor 9, Humans, Idiopathic Pulmonary Fibrosis, Immunohistochemistry, Lung, Myocytes, Smooth Muscle, Myofibroblasts

The fibroblast growth factor (FGF) family of signaling ligands contributes significantly to lung development and maintenance in the adult. FGF9 is involved in control of epithelial branching and mesenchymal proliferation and expansion in developing lungs. However, its activity and expression in the normal adult lung and by epithelial and interstitial cells in fibroproliferative diseases like idiopathic pulmonary fibrosis (IPF) are unknown. Tissue samples from normal organ donor human lungs and those of a cohort of patients with mild to severe IPF were sectioned and stained for the immunolocalization of FGF9. In normal lungs, FGF9 was confined to smooth muscle surrounding airways, alveolar ducts and sacs, and blood vessels. In addition to these same sites, lungs of IPF patients expressed FGF9 in a population of myofibroblasts within fibroblastic foci, hypertrophic and hyperplastic epithelium of airways and alveoli, and smooth muscle cells surrounding vessels embedded in thickened interstitium. The results demonstrate that FGF9 protein increased in regions of active cellular hyperplasia, metaplasia, and fibrotic expansion of IPF lungs, and in isolated human lung fibroblasts treated with TGF-β1 and/or overexpressing Wnt7B. The cellular distribution and established biologic activity of FGF9 make it a potentially strong candidate for contributing to the progression of IPF.

Alternate JournalJ. Histochem. Cytochem.
PubMed ID23797050
PubMed Central IDPMC3753889
Grant ListDK065988 / DK / NIDDK NIH HHS / United States
HL44497 / HL / NHLBI NIH HHS / United States
HL95411 / HL / NHLBI NIH HHS / United States