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Abstract Recent progress in lung biology includes the description of a series of pulmonary stem and progenitor cells involved in homeostasis and regeneration of the respiratory system. Moreover, the contribution of extrapulmonary stem cells to healthy and pathological lung tissue has been observed and the developmental biology of such processes should provide important hints for understanding maintenance and repair of adult lung structure and function. Despite such remarkable advances, the phenotypic and especially the functional characterization of these stem and progenitor cells, and their derivatives, along with an understanding of the molecular cues and pathways underlying differentiation into specific respiratory lineages is still in its infancy. Accordingly, the role of endogenous and extrapulmonary stem cells in normal tissue repair and pathogenesis is still largely mysterious and added basic knowledge is required in order to explore their potential for novel regenerative therapies. This review provides an overview of the current state of the art in adult lung stem cell biology including technical aspects of isolation, characterization and differentiation, and a discussion of perspectives for future regenerative therapies.
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