SFTPC and interstitial lung disease: ,35 Type 2 alveolar epithelial (AT2) cell dysfunction, in particular, has been repeatedly implicated in the pathogenesis of chILD, FPF, and sporadic ILD.36, 37, 38, 39 Based on this new understanding more specific models of FPF are emerging,40, 41, 42 for example a recently developed transgenic mouse model of monogenic lung fibrosis, based on the most common surfactant protein C mutation (SFTPCI73T).43