SFTPC and pulmonary fibrosis: Further, transgenic mice that express the mutant L188Q SP-C did not develop pulmonary fibrosis spontaneously, despite induction of ER stress, but developed a more extensive pulmonary fibrosis with reduced compliance and enhanced AEC apoptosis in response to bleomycin and authors suggested alveolar epithelial ER stress to play a crucial role in enhanced disease development (Lawson et al., 2011).