Disorders of lung matrix remodeling.
A set of lung diseases share the tendency for the development of progressive fibrosis ultimately leading to respiratory failure. This review examines the common pathogenetic features of these disorders in light of recent observations in both humans and animal models of disease, which reveal important pathways of lung matrix remodeling.
Chapman
Harold A
HA
Department of Medicine and Cardiovascular Research Institute, University of California at San Francisco, San Francisco, California 94143-0130, USA. halchap@itsa.ucsf.edu
D000818:Animals; D003094:Collagen / Q000737:chemistry; D004195:Disease Models, Animal; D018450:Disease Progression; D004848:Epithelium / Q000473:pathology; D005355:Fibrosis; D006801:Humans; D007249:Inflammation; D008168:Lung / Q000378:metabolism*; D008171:Lung Diseases / Q000378:metabolism*; D051379:Mice; D008953:Models, Anatomic; D008954:Models, Biological; D009154:Mutation; D011658:Pulmonary Fibrosis / Q000473:pathology*; D015398:Signal Transduction; D016212:Transforming Growth Factor beta / Q000235:genetics / Q000378:metabolism; D053773:Transforming Growth Factor beta1
C508960:TGFB1 protein, human; C508961:Tgfb1 protein, mouse; D016212:Transforming Growth Factor beta; D053773:Transforming Growth Factor beta1; D003094:Collagen