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Approximately 30% of the Abca3<jats:sup>\u0394\/\u0394<\/jats:sup>mice survived after birth. Surviving Abca3<jats:sup>\u0394\/\u0394<\/jats:sup>mice developed emphysema in the absence of significant pulmonary inflammation. Staining of lung tissue and mRNA isolated from alveolar type II cells demonstrated that \u223c50% of alveolar type II cells lacked ABCA3. Phospholipid content and composition were altered in lung tissue, lamellar bodies, and bronchoalveolar lavage fluid from adult Abca3<jats:sup>\u0394\/\u0394<\/jats:sup>mice. In adult Abca3<jats:sup>\u0394\/\u0394<\/jats:sup>mice, cells lacking ABCA3 had decreased expression of mRNAs associated with lipid synthesis and transport. FOXA2 and CCAAT enhancer-binding protein-\u03b1, transcription factors known to regulate genes regulating lung lipid metabolism, were markedly decreased in cells lacking ABCA3. Deletion of Abca3 disrupted surfactant lipid synthesis in a cell-autonomous manner. Compensatory surfactant synthesis was initiated in ABCA3-sufficient type II cells, indicating that surfactant homeostasis is a highly regulated process that includes sensing and coregulation among alveolar type II cells.<\/jats:p>","DOI":"10.1152\/ajplung.00409.2009","type":"journal-article","created":{"date-parts":[[2010,2,27]],"date-time":"2010-02-27T01:54:46Z","timestamp":1267235686000},"page":"L646-L659","source":"Crossref","is-referenced-by-count":54,"title":["Conditional deletion of<i>Abca3<\/i>in alveolar type II cells alters surfactant homeostasis in newborn and adult mice"],"prefix":"10.1152","volume":"298","author":[{"given":"Val\u00e9rie","family":"Besnard","sequence":"first","affiliation":[{"name":"The Perinatal Institute and Section of Neonatology, Perinatal and Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Department of Pediatrics and University of Cincinnati College of Medicine, Cincinnati, 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