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This chapter reviews the current knowledge of the molecular mechanisms controlling embryonic lung development in animal models from the initial specification of a small number of respiratory progenitor cells in the ventral foregut endoderm through the formation of the mature adult lung with regionally specialized epithelial, interstitial, and vascular compartments. In the second half of this chapter we introduce induced pluripotent stem cells (iPSCs) as a compelling new platform to study human lung biology at developmental time-points previously inaccessible to researchers. iPSCs offer the potential to generate functional lung tissue in vitro by translating the knowledge gained from studying respiratory system development in different animal models where many of the signaling pathways or airway branching mechanisms are evolutionarily conserved. There are many exciting possible applications of iPSC-derived lung tissue, including the ability to model human lung disease, screen novel drug therapies, and ultimately generate functional, transplantable lung cells or 3-D tissues for those suffering from one of the many forms of end-stage lung disease.
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