Given that positive SREBP2 staining was observed in lung specimens from human IPF (Figure 7A) and that BLM-induced PF was worsened in EC-SREBP2(N)-Tg mice (Figure 6B), the findings strongly suggest that SREBP2 plays a central role in PF-related lung damage. The gene discussed is SREBF2; the disease is pemphigus foliaceus.