Transforming growth factor-β1 and extracellular matrix-associated fibronectin expression in pulmonary lymphangioleiomyomatosis

SE Evans, TV Colby, JH Ryu, AH Limper - Chest, 2004 - Elsevier
Study objectives Lymphangioleiomyomatosis (LAM) is a rare disorder of unknown etiology,
affecting almost exclusively women of childbearing age, that is associated with the
proliferation of spindle cells and cystic changes in the affected lung. The underlying
processes that contribute to this disease are poorly understood. Transforming growth factor
(TGF)-β 1 is a potent cytokine that promotes mesenchymal cell proliferation and regulates
the synthesis of extracellular matrix (ECM) components, particularly fibronectins. Herein, we …