NDLI: Long-chain acyl-CoA dehydrogenase deficiency as a cause of pulmonary surfactant dysfunction.

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Long-chain acyl-CoA dehydrogenase deficiency as a cause of pulmonary surfactant dysfunction.

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Long-chain acyl-CoA dehydrogenase deficiency as a cause of pulmonary surfactant dysfunction.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Karunanidhi, Anuradha Kagan, Valerian E. Goetzman, Eric S. Kosmider, Beata Zuo, Yi Y. Otsubo, Chikara Mallampalli, Rama K. Skilling, Helen Chen, Rimei Suhrie, Kristen R. Uppala, Radha Mchugh, Kevin J. Ellis, Bryon Yeasted, Renita Tyurina, Yulia Y. Mckinney, Richard W. Alcorn, John F. Beck, Megan E. Bharathi, Sivakama S. Vockley, Jerry
Description	Author Affiliation: Goetzman ES ( From the Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh of University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15224.)
Abstract	Long-chain acyl-CoA dehydrogenase (LCAD) is a mitochondrial fatty acid oxidation enzyme whose expression in humans is low or absent in organs known to utilize fatty acids for energy such as heart, muscle, and liver. This study demonstrates localization of LCAD to human alveolar type II pneumocytes, which synthesize and secrete pulmonary surfactant. The physiological role of LCAD and the fatty acid oxidation pathway in lung was subsequently studied using LCAD knock-out mice. Lung fatty acid oxidation was reduced in LCAD(-/-) mice. LCAD(-/-) mice demonstrated reduced pulmonary compliance, but histological examination of lung tissue revealed no obvious signs of inflammation or pathology. The changes in lung mechanics were found to be due to pulmonary surfactant dysfunction. Large aggregate surfactant isolated from LCAD(-/-) mouse lavage fluid had significantly reduced phospholipid content as well as alterations in the acyl chain composition of phosphatidylcholine and phosphatidylglycerol. LCAD(-/-) surfactant demonstrated functional abnormalities when subjected to dynamic compression-expansion cycling on a constrained drop surfactometer. Serum albumin, which has been shown to degrade and inactivate pulmonary surfactant, was significantly increased in LCAD(-/-) lavage fluid, suggesting increased epithelial permeability. Finally, we identified two cases of sudden unexplained infant death where no lung LCAD antigen was detectable. Both infants were homozygous for an amino acid changing polymorphism (K333Q). These findings for the first time identify the fatty acid oxidation pathway and LCAD in particular as factors contributing to the pathophysiology of pulmonary disease.
ISSN	00219258
e-ISSN	1083351X
Journal	Journal of Biological Chemistry
Issue Number	15
Volume Number	289
Language	English
Publisher	American Society for Biochemistry and Molecular Biology (United States)
Publisher Date	2014-04-11
Publisher Place	United States
Access Restriction	Open
Subject Keyword	Acyl-CoA Dehydrogenase, Long-Chain Deficiency Lipid Metabolism, Inborn Errors Metabolism Lung Diseases Etiology Pulmonary Surfactants Animals Bronchi Cell Line, Tumor Coenzyme A Disease Models, Animal Epithelial Cells Fatty Acids Homozygote Infant Infant, Newborn Lung Lung Neoplasms Mice Mice, Inbred C57BL Mice, Transgenic Oxygen Phosphatidylcholines Chemistry Phosphatidylglycerols Polymorphism, Genetic Pulmonary Alveoli Research Support, N.I.H., Extramural Research Support, U.S. Gov't, Non-P.H.S. Biochemistry Molecular Biology
Content Type	Text
Resource Type	Article
Subject	Cell Biology Biochemistry Molecular Biology

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