Pulmonary endothelial injury in the context of perturbed transforming growth factor β signalling as a unique model of pulmonary hypertension in scleroderma

Fibroblast-specific perturbation of tran

Fibroblast-specific perturbation of transforming growth factor β signaling provides insight into potential pathogenic mechanisms of scleroderma-associated lung fibrosis: Exaggerated response to alveolar epithelial injury in a novel mouse model

✍ Rachel K. Hoyles; Korsa Khan; Xu Shiwen; Sarah L. Howat; Gisela E. Lindahl; Patr 📂 Article 📅 2008 🏛 John Wiley and Sons 🌐 English ⚖ 860 KB

## Abstract ## Objective To explore increased susceptibility to fibrosis following experimental injury to alveolar epithelial cells (AECs) in a novel transgenic mouse model of scleroderma with fibroblast‐specific perturbation of transforming growth factor β (TGFβ) signaling (TβRIIΔk‐fib mice). ##