At 28 days post 3-dose LPS challenge, obvious scars were observed in Ism1−/− lung (Additional file 1: Fig. S6b). Excessive accumulation of extracellular matrix (ECM), myofibroblasts and scar formation are characteristics of pulmonary fibrosis (Todd et al. 2012). The gene discussed is ISM1; the disease is pulmonary fibrosis.