Imbalanced expression of the type I TGF-beta receptors ALK-1 and ALK-5 in pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a devastating disease with no effective treatment options. The lungs of IPF patients are characterized by alveolar epithelial cell injury, an accumulation of activated (myo)fibroblasts, and excessive deposition of extracellular matrix. This is, to a great extend, due to enhanced TGF-beta activity. In the study, we hypothesized that IPF is characterized by distinct alterations in the expression and activity of TGF-beta receptors (TbR) and its intracellular mediators, smads. We analyzed TbR and smad1–9 expression in human lung homogenates derived from IPF patients (n=12) and…