Moreover, Notch3 expression was regulated by nestin in vitro and vivo; nestin gene silencing resulted in reduced Notch3 expression and enhanced collagen expression and EndoMT in vitro and vivo. Contrary to previous reports, both Nes mRNA expression in nestin-expressing cells as well as Notch3-expressing cells were decreased during pulmonary fibrosis, and a significant inverse correlation between nestin-expressing cells and collagen production was observed. Here, NOTCH3 is linked to pulmonary fibrosis.