Impaired Distal Airway Development in Mice Lacking Elastin

Elastin is a major component of the mammalian lung, predominantly found in the alveoli. Destruction of alveolar elastic fibers is implicated in the pathogenic mechanism of emphysema in adults. These data define a role for elastin in the structure and function of the mature lung, and suggest that ela...

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Published inAmerican journal of respiratory cell and molecular biology Vol. 23; no. 3; pp. 320 - 326
Main Authors Wendel, Daniel P, Taylor, Douglas G, Albertine, Kurt H, Keating, Mark T, Li, Dean Y
Format Journal Article
LanguageEnglish
Published United States Am Thoracic Soc 01.09.2000
Subjects
Actins - analysis
Animals
Animals, Newborn
Gene Expression Regulation, Developmental - physiology
Mesoderm - physiology
Mice
Mice, Inbred C57BL
Mice, Knockout
Phenotype
Pulmonary Alveoli - abnormalities
Pulmonary Alveoli - chemistry
Pulmonary Alveoli - embryology
Tropoelastin - analysis
Tropoelastin - genetics
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Summary:Elastin is a major component of the mammalian lung, predominantly found in the alveoli. Destruction of alveolar elastic fibers is implicated in the pathogenic mechanism of emphysema in adults. These data define a role for elastin in the structure and function of the mature lung, and suggest that elastin is important for alveogenesis. To investigate the role of elastin in lung development, we examined mice lacking elastin (Eln-/-). At birth, the distal air sacs of Eln-/- lungs dilate to form abnormally large cavities. This phenotype appears before the synthesis and deposition of alveolar elastin, a process mediated by myofibroblasts and initiated after postnatal Day 4. Morphometric analyses demonstrate that the perinatal development of terminal airway branches is arrested in Eln-/- mice. The branching defect is accompanied by fewer distal air sacs that are dilated with attenuated tissue septae, a condition reminiscent of emphysema. Elastin expression in the lung parenchyma before alveogenesis is localized to the mesenchyme surrounding the developing airways, supporting a role for elastin in airway branching. Thus, in addition to its role in the structure and function of the mature lung, elastin is essential for pulmonary development and is important for terminal airway branching.
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ISSN:1044-1549
1535-4989
DOI:10.1165/ajrcmb.23.3.3906