Modulation of Myofibroblast and Smooth-Muscle Phenotypes in the Lung

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Abstract
The lung contains a full complement of vascular and visceral smooth-muscle cells (SMCs) [2, 16, 18]. Additionally, in the normal alveolar wall, there are contractile interstitial cells that contribute to normal function [10, 12] as well as to alveolar remodeling seen in such disorders as pulmonary hypertension and interstitial fibrosis [11, 21]. This review will present three topics: (1) the differentiation of these cells during normal lung development, (2) the phenotypic changes that occur during pulmonary interstitial remodeling, including the appearance of alveolar and interstitial myofibroblasts during pulmonary fibrosis, and (3) the regulation of cell phenotype by transforming growth factor-β (TGF-β). Other chapters in this volume also are particularly relevant to what is covered here (Chaps. 1, 5, 6, 15, 18).