Alterations in SP-B and SP-C Expression in Neonatal Lung Disease
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- 1 March 2004
- journal article
- review article
- Published by Annual Reviews in Annual Review of Physiology
- Vol. 66 (1) , 601-623
- https://doi.org/10.1146/annurev.physiol.66.032102.134711
Abstract
▪ Abstract The hydrophobic surfactant proteins, SP-B and SP-C, have important roles in surfactant function. The importance of these proteins in normal lung function is highlighted by the lung diseases associated with abnormalities in their expression. Mutations in the gene encoding SP-B result in severe, fatal neonatal lung disease, and mutations in the gene encoding SP-C are associated with chronic interstitial lung diseases in newborns, older children, and adults. This work reviews the current state of knowledge concerning the lung diseases associated with mutations in the SP-B and SP-C genes, and the potential roles of abnormal SP-B and SP-C expression and genetic variation in these genes in other lung diseases.Keywords
This publication has 147 references indexed in Scilit:
- Surfactant protein B polymorphism and respiratory distress syndrome in premature twinsHuman Genetics, 2003
- The palmitoyl groups of lung surfactant protein C reduce unfolding into a fibrillogenic intermediateJournal of Molecular Biology, 2001
- Lung transplantation for treatment of infants with surfactant protein B deficiencyThe Journal of Pediatrics, 1997
- Surfactant proteins and stable microbubbles in tracheal aspirates of infants with respiratory distress syndrome: relation to the degree of respiratory failure and response to exogenous surfactantEuropean Journal of Pediatrics, 1997
- On the surface activity of surfactant-associated protein C (SP-C): Effects of palmitoylation and pHBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1995
- Surfactant protein B deficiency: Antenatal diagnosis and prospective treatment with surfactant replacementThe Journal of Pediatrics, 1994
- Molecular and phenotypic variability in the congenital alveolar proteinosis syndrome associated with inherited surfactant protein B deficiencyThe Journal of Pediatrics, 1994
- Deficiency of Pulmonary Surfactant Protein B in Congenital Alveolar ProteinosisNew England Journal of Medicine, 1993
- DNA base changes and RNA levels in N-acetoxy-2-acetylaminofluorene-induced dihydrofolate reductase mutants of Chinese hamster ovary cellsJournal of Molecular Biology, 1989
- cDNA and deduced amino acid sequence for the rat hydrophobic pulmonary surfactant-associated protein, SP-BBiochimica et Biophysica Acta (BBA) - Protein Structure and Molecular Enzymology, 1989