Expression of fibroblast growth factor 9 in normal human lung and idiopathic pulmonary fibrosis.
J Histochem Cytochem
; 61(9): 671-9, 2013 Sep.
Article
en En
| MEDLINE
| ID: mdl-23797050
ABSTRACT
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-ß1 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.
Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Factor 9 de Crecimiento de Fibroblastos
/
Fibrosis Pulmonar Idiopática
/
Pulmón
Límite:
Adult
/
Humans
Idioma:
En
Revista:
J Histochem Cytochem
Asunto de la revista:
HISTOCITOQUIMICA
Año:
2013
Tipo del documento:
Article
País de afiliación:
Estados Unidos