The molecular genetics of lung morphogenesis and injury repair.

摘要: Abstract Lung development, as well epithelial injury repair, is tightly coordinated by a fine balance between stimulatory versus inhibitory genes that appear to co-regulate the function of stem/progenitor cells in lung. Recently, it has been noted many same direct development respiratory organs (tracheae) fruit fly Drosophila mice and men. For example, FGF receptor tyrosine kinase signaling essential for organogenesis both mouse negatively regulated sprouty genes, family inducible pathway inhibitors. Additionally, required formation new alveoli, protection alveolar from injury, migration proliferation putative during lung repair. Conversely, TGFβ serine-threonine via Smads 2, 3 4 inhibits morphogenesis can inhibit postnatal while excessive Smad3 causes interstitial fibrosis. On other hand, BMP4 stimulates intact embryonic lung, inhibiting isolated epithelium. We speculate evolutionary-developmental, functional conservation FGF- FGFR-SPROUTY TGFβ/BMP-SMAD modulatory pathways identifies them potential therapeutic targets rational therapy. Novel therapy activate cells, ameliorate augment repair and/or induce regeneration could be highly beneficial children adults with intractable pulmonary insufficiency.