Elastin in lung development and disease.
Elastic fibres are present in lung structures including alveoli, alveolar ducts, airways, vasculature and pleura. The rate of lung elastin synthesis is greatest during fetal and neonatal development, and is minimal in the healthy adult. We have determined that glucocorticoids up-regulate fetal lung tropoelastin expression while concomitantly accelerating terminal airspace maturation. Because there is minimal turnover of elastin in healthy adult lung, the elastin incorporated into the lung early in development supports lung function for the normal lifespan. However, in the adult lung, in pathological circumstances such as emphysema or pulmonary fibrosis there may be reactivation of elastin expression. We have found in silica-induced pulmonary fibrosis that expression of tropoelastin is primarily increased in the walls and the septal tips of the alveolus, with modest increases in other compartments which normally express tropoelastin during development. This finding suggests that the mesenchymal cell of the alveolar wall increases tropoelastin expression during fibrotic disorders. In emphysema and fibrosis, elastin is present in abnormal-appearing, probably non-functional, elastic fibres, suggesting that the adult lung cannot recapitulate the elastic fibre assembly mechanisms operative during normal lung growth.
Pierce
R A
RA
Division of Dermatology, Washington University School of Medicine, Jewish Hospital, St. Louis, MO 63110, USA.
Mariani
T J
TJ
Senior
R M
RM
D000328:Adult; D000818:Animals; D004549:Elastin / Q000235:genetics / Q000378:metabolism*; D005333:Fetus; D006801:Humans; D008168:Lung / Q000196:embryology / Q000254:growth & development / Q000378:metabolism*; D011656:Pulmonary Emphysema / Q000378:metabolism*; D011658:Pulmonary Fibrosis / Q000378:metabolism*
D004549:Elastin