Role of sphingolipids in the pathobiology of lung inflammation

Mediators of Inflammation

Volumn 2015, Issue , 2015, Pages

  Ghidoni, Riccardo ^a   Caretti, Anna ^a   Signorelli, Paola ^a  

^a UNIVERSITY OF MILAN   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

CERAMIDE; LACTOSYLCERAMIDE; LUNG SURFACTANT; SPHINGOLIPID; CFTR PROTEIN, HUMAN; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR;

ACUTE LUNG INJURY; ADULT RESPIRATORY DISTRESS SYNDROME; BLOOD VESSEL PERMEABILITY; BRONCHOCONSTRICTION; CELL PHAGOCYTOSIS; CHRONIC INFLAMMATION; CHRONIC OBSTRUCTIVE LUNG DISEASE; CYSTIC FIBROSIS; DISEASE ASSOCIATION; ENVIRONMENTAL STRESS; FETUS LUNG MATURATION; GENETIC DISORDER; HUMAN; INTRACELLULAR SIGNALING; LIPID METABOLISM; LUNG ALVEOLUS MACROPHAGE; LUNG EDEMA; LUNG ENDOTHELIUM; LUNG FIBROSIS; LUNG FUNCTION; LUNG GAS EXCHANGE; LUNG PARENCHYMA; LUNG STRUCTURE; NONHUMAN; PATHOGENESIS; PNEUMONIA; PRIORITY JOURNAL; REVIEW; THIN LAYER CHROMATOGRAPHY; VASOCONSTRICTION; ANIMAL; MOUSE; PATHOPHYSIOLOGY; PHYSIOLOGY;

ANIMALS; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; HUMANS; MICE; PNEUMONIA; PULMONARY DISEASE, CHRONIC OBSTRUCTIVE; SPHINGOLIPIDS;

EID: 84950118332     PISSN: 09629351     EISSN: 14661861     Source Type: Journal    
DOI: 10.1155/2015/487508     Document Type: Review

References (152)

1. I. Petrache and D. N. Petrusca, "The involvement of sphingolipids in chronic obstructive pulmonary diseases," Handbook of Experimental Pharmacology, vol. 216, pp. 247-264, 2013.
2. J. Tibboel, I. Reiss, J. C. de Jongste, and M. Post, "Sphingolipids in lung growth and repair," Chest, vol. 145, no. 1, pp. 120-128, 2014.
3. S. Uhlig and Y. Yang, "Sphingolipids in acute lung injury," Handbook of Experimental Pharmacology, vol. 216, pp. 227-246, 2013.
4. Y. Yang and S. Uhlig, "The role of sphingolipids in respiratory disease,"Therapeutic Advances in Respiratory Disease, vol. 5, no. 5, pp. 325-344, 2011.
5. L. Gluck, E. K. Motoyama, H. L. Smits, and M.V. Kulovich, "The biochemical development of surface activity in mammalian lung. I. The surface-active phospholipids; the separation and distribution of surface-active lecithin in the lung of the developing rabbit fetus," Pediatric Research, vol. 1, no. 4, pp. 237-246, 1967.
6. S. J. Thannhauser, J. Penotti, and N. F. Boncoddo, "Isolation and properties of hydrolecithin (dipalmityl lecithin) from lung; its occurrence in the sphingomyelin fraction of animal tissues," The Journal of Biological Chemistry, vol. 166, no. 2, pp. 669-675, 1946.
7. L. Gluck, M. V. Kulovich, R. C. Borer Jr., P. H. Brenner, G. G. Anderson, and W. N. Spellacy, "Diagnosis of the respiratory distress syndrome by amniocentesis," American Journal of Obstetrics and Gynecology, vol. 109, no. 3, pp. 440-445, 1971.
8. T. A. Doran, J. A. Ford, L. C. Allen, P. Y. Wong, and R. J. Benzie, "Amniotic fluid lecithin/sphingomyelin ratio, palmitic acid, palmitic acid/stearic acid ratio, total cortisol, creatinine, and percentage of lipid-positive cells in assessment of fetal maturity and fetal pulmonarymaturity: a comparison,"American Journal of Obstetrics and Gynecology, vol. 133, no. 3, pp. 302-307, 1979.
9. A. E. Besnard, S. A. M. Wirjosoekarto, K. A. Broeze, B. C. Opmeer, and B. W. J. Mol, "Lecithin/sphingomyelin ratio and lamellar body count for fetal lung maturity: a meta-analysis," European Journal of Obstetrics Gynecology and Reproductive Biology, vol. 169, no. 2, pp. 177-183, 2013.
10. M.Hallman and L. Gluck, "Formation of acidic phopholipids in rabbit lung during perinatal development," Pediatric Research, vol. 14, no. 11, pp. 1250-1259, 1980.
11. C. A. Longo, D. Tyler, and R. K. Mallampalli, "Sphingomyelin metabolism is developmentally regulated in rat lung," American Journal of Respiratory Cell and Molecular Biology, vol. 16, no. 5, pp. 605-612, 1997.
12. T. A. Lagace and N. D. Ridgway, "The role of phospholipids in the biological activity and structure of the endoplasmic reticulum," Biochimica et Biophysica Acta, vol. 1833, no. 11, pp. 2499-2510, 2013.
13. A. H. Merrill Jr., D.W. Nixon, and R. D. Williams, "Activities of serine palmitoyltransferase (3-ketosphinganine synthase) in microsomes fromdifferent rat tissues," Journal of Lipid Research, vol. 26, no. 5, pp. 617-622, 1985.
14. A. J. Westover and T. J. M. Moss, "Effects of intrauterine infection or inflammation on fetal lung development," Clinical and Experimental Pharmacology and Physiology, vol. 39, no. 9, pp. 824-830, 2012.
15. M. Hallman, "The surfactant system protects both fetus and newborn," Neonatology, vol. 103, no. 4, pp. 320-326, 2013.
16. H. Khammash, M. Perlman, J. Wojtulewicz, and M. Dunn, "Surfactant therapy in full-term neonates with severe respiratory failure," Pediatrics, vol. 92, no. 1, pp. 135-139, 1993.
17. R. L. Auten, R. H. Notter, J. W. Kendig, J. M. Davis, and D. L. Shapiro, "Surfactant treatment of full-term newborns with respiratory failure," Pediatrics, vol. 87, no. 1, pp. 101-107, 1991.
18. V. Zambelli, G. Bellani, M. Amigoni et al., "The effects of exogenous surfactant treatment in a murine model of two-hit lung injury," Anesthesia and Analgesia, vol. 120, no. 2, pp. 381-388, 2015.
19. M. P. Sherman, J. B. DAmbola, E. E. Aeberhard, and C. T. Barrett, "Surfactant therapy of newborn rabbits impairs lung macrophage bactericidal activity," Journal of Applied Physiology, vol. 65, no. 1, pp. 137-145, 1988.
20. I. Bersani, S. Kunzmann, and C. P. Speer, "Immunomodulatory properties of surfactant preparations," Expert Review of Anti-InfectiveTherapy, vol. 11, no. 1, pp. 99-110, 2013.
21. M. Nishimura, T. Saida, S. Kuroki et al., "Post-infectious encephalitis with anti-galactocerebroside antibody subsequent to Mycoplasma pneumoniae infection," Journal of the Neurological Sciences, vol. 140, no. 1-2, pp. 91-95, 1996.
22. S. de Bentzmann, P. Roger, and E. Puchelle, "Pseudomonas aeruginosa adherence to remodelling respiratory epithelium," The European Respiratory Journal, vol. 9, no. 10, pp. 2145-2150, 1996.
23. A. J. Ratner, R. Bryan, A. Weber et al., "Cystic fibrosis pathogens activate Ca2+-dependent mitogen-activated protein kinase signaling pathways in airway epithelial cells," The Journal of Biological Chemistry, vol. 276, no. 22, pp. 19267-19275, 2001.
24. M. Gotoh, S. Tsuru, N. Shinomiya et al., "Functional changes of alveolar macrophages in carragheenan-induced aspiration pneumonia model mice," Natural Immunity, vol. 11, no. 6, pp. 345-355, 1992.
25. M. M. B. Moreno-Altamirano, I. Aguilar-Carmona, and F. J. Sánchez-García, "Expression of GM1, a marker of lipid rafts, defines two subsets of human monocytes with differential endocytic capacity and lipopolysaccharide responsiveness," Immunology, vol. 120, no. 4, pp. 536-543, 2007.
26. C. Chan and T. Goldkorn, "Ceramide path in human lung cell death," American Journal of Respiratory Cell and Molecular Biology, vol. 22, no. 4, pp. 460-468, 2000.
27. S. N. Lavrentiadou, C. Chan, T. Kawcak et al., "Ceramidemediated apoptosis in lung epithelial cells is regulated by glutathione,"American Journal of Respiratory Cell andMolecular Biology, vol. 25, no. 6, pp. 676-684, 2001.
28. S. Filosto, S. Castillo, A. Danielson et al., "Neutral sphingomyelinase 2: a novel target in cigarette smoke-induced apoptosis and lung injury," American Journal of Respiratory Cell andMolecular Biology, vol. 44, no. 3, pp. 350-360, 2011.
29. T. Goldkorn, T. Ravid, and E. M. Khan, "Life and death decisions: ceramide generation and EGF receptor trafficking are modulated by oxidative stress," Antioxidants and Redox Signaling, vol. 7, no. 1-2, pp. 119-128, 2005.
30. E. Abraham, "Neutrophils and acute lung injury," Critical Care Medicine, vol. 31, pp. S195-S199, 2003.
31. M. Levy, S. S. Castillo, and T. Goldkorn, "nSMase2 activation and trafficking are modulated by oxidative stress to induce apoptosis," Biochemical and Biophysical Research Communications, vol. 344, no. 3, pp. 900-905, 2006.
32. S. S. Castillo, M. Levy, C. Wang, J. V. Thaikoottathil, E. Khan, and T. Goldkorn, "Nitric oxide-enhanced caspase-3 and acidic sphingomyelinase interaction: a novel mechanism by which airway epithelial cells escape ceramide-induced apoptosis," Experimental Cell Research, vol. 313, no. 4, pp. 816-823, 2007.
33. B. J. Pettus, C. E. Chalfant, and Y. A. Hannun, "Sphingolipids in inflammation: roles and implications," Current Molecular Medicine, vol. 4, no. 4, pp. 405-418, 2004.
34. S. S. Castillo, M. Levy, J. V. Thaikoottathil, and T. Goldkorn, "Reactive nitrogen and oxygen species activate different sphingomyelinases to induce apoptosis in airway epithelial cells," Experimental Cell Research, vol. 313, no. 12, pp. 2680-2686, 2007.
35. J. G. N. Garcia, F. Liu, A. D. Verin et al., "Sphingosine 1-phosphate promotes endothelial cell barrier integrity by Edgdependent cytoskeletal rearrangement," The Journal of Clinical Investigation, vol. 108, no. 5, pp. 689-701, 2001.
36. M. Maceyka and S. Spiegel, "Sphingolipidmetabolites in inflammatory disease," Nature, vol. 510, no. 7503, pp. 58-67, 2014.
37. J. D. Saba and T. Hla, "Point-counterpoint of sphingosine 1-phosphate metabolism," Circulation Research, vol. 94, no. 6, pp. 724-734, 2004.
38. S. Spiegel and S. Milstien, "Sphingosine-1-phosphate: an enigmatic signalling lipid," Nature Reviews Molecular Cell Biology, vol. 4, no. 5, pp. 397-407, 2003.
39. A. Abe, M. Hiraoka, S. Wild, S. E. Wilcoxen, R. Paine III, and J. A. Shayman, "Lysosomal phospholipase A2 is selectively expressed in alveolar macrophages," The Journal of Biological Chemistry, vol. 279, no. 41, pp. 42605-42611, 2004.
40. Y. Zhao, S. K. Kalari, P. V. Usatyuk et al., "Intracellular generation of sphingosine 1-phosphate in human lung endothelial cells: role of lipid phosphate phosphatase-1 and sphingosine kinase 1," The Journal of Biological Chemistry, vol. 282, no. 19, pp. 14165-14177, 2007.
41. T. Abbasi and J. G.N. Garcia, "Sphingolipids in lung endothelial biology and regulation of vascular integrity," Handbook of Experimental Pharmacology, vol. 216, pp. 201-226, 2013.
42. Y. Kasahara, R. M. Tuder, L. Taraseviciene-Stewart et al., "Inhibition of VEGF receptors causes lung cell apoptosis and emphysema," The Journal of Clinical Investigation, vol. 106, no. 11, pp. 1311-1319, 2000.
43. K. J. Diab, J. J. Adamowicz, K. Kamocki et al., "Stimulation of sphingosine 1-phosphate signaling as an alveolar cell survival strategy in emphysema," American Journal of Respiratory and Critical Care Medicine, vol. 181, no. 4, pp. 344-352, 2010.
44. I. Petrache, V. Natarajan, L. Zhen et al., "Ceramide upregulation causes pulmonary cell apoptosis and emphysema-like disease in mice," Nature Medicine, vol. 11, no. 5, pp. 491-498, 2005.
45. S. Mandala, R. Hajdu, J. Bergstrom et al., "Alteration of lymphocyte trafficking by sphingosine-1-phosphate receptor agonists," Science, vol. 296, no. 5566, pp. 346-349, 2002.
46. L. Wang, S. Sammani, L. Moreno-Vinasco et al., "FTY720 (S)-phosphonate preserves sphingosine 1-phosphate receptor 1 expression and Exhibits superior barrier protection to FTY720 in acute lung injury," Critical Care Medicine, vol. 42, no. 3, pp. e189-e199, 2014.
47. L. O. Myat, S. Thangada, M.-T. Wu et al., "Immunosuppressive and anti-angiogenic sphingosine 1-phosphate receptor-1 agonists induce ubiquitinylation and proteasomal degradation of the receptor,"The Journal of Biological Chemistry, vol. 282, no. 12, pp. 9082-9089, 2007.
48. E. V. Berdyshev, I. Gorshkova, A. Skobeleva et al., "FTY720 inhibits ceramide synthases and up-regulates dihydrosphingosine 1-phosphate formation in human lung endothelial cells," The Journal of Biological Chemistry, vol. 284, no. 9, pp. 5467-5477, 2009.
49. R. T. Dobrowsky, C. Kamibayashi, M. C. Mumby, and Y.A. Hannun, "Ceramide activates heterotrimeric protein phosphatase 2A," Journal of Biological Chemistry, vol. 268, no. 21, pp. 15523-15530, 1993.
50. A.Mukhopadhyay, S. A. Saddoughi, P. Song et al., "Direct interaction between the inhibitor 2 and ceramide via sphingolipidprotein binding is involved in the regulation of protein phosphatase 2A activity and signaling," The FASEB Journal, vol. 23, no. 3, pp. 751-763, 2009.
51. T. T. Cornell, V. Hinkovska-Galcheva, L. Sun et al., "Ceramidedependent PP2A regulation of TNFα-induced IL-8 production in respiratory epithelial cells," American Journal of Physiology- Lung Cellular and Molecular Physiology, vol. 296, no. 5, pp. L849-L856, 2009.
52. K. Baudiß, C. K. Ayata, Z. Lazar et al., "Ceramide-1-phosphate inhibits cigarette smoke-induced airway inflammation," The European Respiratory Journal, vol. 45,no. 6, pp. 1669-1680, 2015.
53. D. Avni, A. Philosoph, M. M. Meijler, and T. Zor, "The ceramide-1-phosphate analogue PCERA-1modulates tumour necrosis factor-α and interleukin-10 production in macrophages via the cAMP-PKA-CREB pathway in a GTP-dependent manner," Immunology, vol. 129, no. 3, pp. 375-385, 2010.
54. S. Uhlig, R. Göggel, and S. Engel, "Mechanisms of plateletactivating factor (PAF)-mediated responses in the lung," Pharmacological Reports, vol. 57, supplement, pp. 206-221, 2006.
55. W. M. Kuebler, Y. Yang, R. Samapati, and S. Uhlig, "Vascular barrier regulation by PAF, ceramide, caveolae, and NO- an intricate signaling network with discrepant effects in the pulmonary and systemic vasculature," Cellular Physiology and Biochemistry, vol. 26, no. 1, pp. 29-40, 2010.
56. J. Balsinde, M. A. Balboa, and E. A. Dennis, "Inflammatory activation of arachidonic acid signaling in murine P388D1 macrophages via sphingomyelin synthesis," The Journal of Biological Chemistry, vol. 272, no. 33, pp. 20373-20377, 1997.
57. R. Newton, L. Hart, K. F. Chung, and P. J. Barnes, "Ceramide induction of COX-2 and PGE2 in pulmonary A549 cells does not involve activation of NF-κB," Biochemical and Biophysical Research Communications, vol. 277, no. 3, pp. 675-679, 2000.
58. W. Stremmel, A. Hanemann, A. Braun et al., "Delayed release phosphatidylcholine as new therapeutic drug for ulcerative colitis-a review of three clinical trials," Expert Opinion on Investigational Drugs, vol. 19, no. 12, pp. 1623-1630, 2010.
59. E. Feige, I. Mendel, J. George, N. Yacov, and D. Harats, "Modified phospholipids as anti-inflammatory compounds," Current Opinion in Lipidology, vol. 21, no. 6, pp. 525-529, 2010.
60. J. Vivekananda, D. Smith, and R. J. King, "Sphingomyelin metabolites inhibit sphingomyelin synthase and CTP: phosphocholine cytidylyltransferase," The American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 281, no. 1, pp. L98-L107, 2001.
61. S. Awasthi, J. Vivekananda, V. Awasthi, D. Smith, and R. J. King, "CTP:Phosphocholine cytidylyltransferase inhibition by ceramide via PKC-α, p38 MAPK, cPLA2, and 5-lipoxygenase," American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 281, no. 1, pp. L108-L118, 2001.
62. M. Murakami and I. Kudo, "New phospholipase A2 isozymes with a potential role in atherosclerosis," Current Opinion in Lipidology, vol. 14, no. 5, pp. 431-436, 2003.
63. Z. Xu, J. Zhou, D. M. McCoy, and R. K. Mallampalli, "LASS5 is the predominant ceramide synthase isoform involved in de novo sphingolipid synthesis in lung epithelia," Journal of Lipid Research, vol. 46, no. 6, pp. 1229-1238, 2005.
64. A. J. Ryan, D. M. McCoy, S. E. McGowan, R. G. Salome, and R. K. Mallampalli, "Alveolar sphingolipids generated in response to TNF-α modifies surfactant biophysical activity," Journal of Applied Physiology, vol. 94, no. 1, pp. 253-258, 2003.
65. P. Miklavc, O. H. Wittekindt, E. Felder, and P. Dietl, "Ca2+-dependent actin coating of lamellar bodies after exocytotic fusion: a prerequisite for content release or kiss-and-run," Annals of the New York Academy of Sciences, vol. 1152, pp. 43-52, 2009.
66. L. Sparkman, H. Chandru, and V. Boggaram, "Ceramide decreases surfactant protein B gene expression via downregulation of TTF-1 DNA binding activity," American Journal of Physiology: Lung Cellular andMolecular Physiology, vol. 290, no. 2, pp. L351-L358, 2006.
67. A. H. Merrill Jr., E. Wang, J. Stevens, and G. W. Brumley, "Activities of the initial enzymes of glycerolipid and sphingolipid synthesis in lung microsomes from rats exposed to air or 85% oxygen," Biochemical and Biophysical Research Communications, vol. 119, no. 3, pp. 995-1000, 1984.
68. N. Kolliputi, L. Galam, P. T. Parthasarathy, S. M. Tipparaju, and R. F. Lockey, "NALP-3 inflammasome silencing attenuates ceramide-induced transepithelial permeability," Journal of Cellular Physiology, vol. 227, no. 9, pp. 3310-3316, 2012.
69. J. Tibboel, S. Joza, I. Reiss, J. C. de Jongste, andM. Post, "Amelioration of hyperoxia-induced lung injury using a sphingolipidbased intervention," The European Respiratory Journal, vol. 42, no. 3, pp. 776-784, 2013.
70. S. Yu, M. Shi, C. Liu et al., "Time course changes of oxidative stress and inflammation in hyperoxia-induced acute lung injury in rats," Iranian Journal of Basic Medical Sciences, vol. 18, no. 1, pp. 98-103, 2015.
71. H. Rauvala and M. Hallman, "Glycolipid accumulation in bronchoalveolar space in adult respiratory distress syndrome," Journal of Lipid Research, vol. 25, no. 11, pp. 1257-1262, 1984.
72. W. Drobnik, G. Liebisch, F.-X. Audebert et al., "Plasma ceramide and lysophosphatidylcholine inversely correlate with mortality in sepsis patients," Journal of Lipid Research, vol. 44, no. 4, pp. 754-761, 2003.
73. R. Göggell, S. Winoto-Morbach, G. Vielhaber et al., "PAFmediated pulmonary edema: a new role for acid sphingomyelinase and ceramide," Nature Medicine, vol. 10, no. 2, pp. 155-160, 2004.
74. P. von Bismarck, C.-F. G. Wistädt, K. Klemm et al., "Improved pulmonary function by acid sphingomyelinase inhibition in a newborn piglet lavage model," American Journal of Respiratory and Critical Care Medicine, vol. 177, no. 11, pp. 1233-1241, 2008.
75. R. Dhami, X.He, and E. H. Schuchman, "Acid sphingomyelinase deficiency attenuates bleomycin-induced lung inflammation and fibrosis in mice," Cellular Physiology and Biochemistry, vol. 26, no. 4-5, pp. 749-760, 2010.
76. J. Chen, H. Tang, J. R. Sysol et al., "The sphingosine kinase 1/sphingosine-1-phosphate pathway in pulmonary arterial hypertension," American Journal of Respiratory and Critical Care Medicine, vol. 190, no. 9, pp. 1032-1043, 2014.
77. V. Natarajan, S. M. Dudek, J. R. Jacobson et al., "Sphingosine-1-phosphate, FTY720, and sphingosine-1-phosphate receptors in the pathobiology of acute lung injury," American Journal of Respiratory Cell and Molecular Biology, vol. 49, no. 1, pp. 6-17, 2013.
78. B. Mathew, J. R. Jacobson, E. Berdyshev et al., "Role of sphingolipids inmurine radiation-induced lung injury: protection by sphingosine 1-phosphate analogs," The FASEB Journal, vol. 25, no. 10, pp. 3388-3400, 2011.
79. W.-C. Lin, C.-F. Lin, C.-L. Chen, C.-W. Chen, and Y.-S. Lin, "Inhibition of neutrophil apoptosis via sphingolipid signaling in acute lung injury," The Journal of Pharmacology and Experimental Therapeutics, vol. 339, no. 1, pp. 45-53, 2011.
80. S. Ren, C. Xin, J. Pfeilschifter, and A. Huwiler, "A novel mode of action of the putative sphingosine kinase inhibitor 2-(phydroxyanilino)-4-(p-chlorophenyl) thiazole (SKI II): induction of lysosomal sphingosine kinase 1 degradation," Cellular Physiology and Biochemistry, vol. 26, no. 1, pp. 97-104, 2010.
81. W. P. R. Verdurmen, M. Thanos, I. R. Ruttekolk, E. Gulbins, and R. Brock, "Cationic cell-penetrating peptides induce ceramide formation via acid sphingomyelinase: implications for uptake," Journal of Controlled Release, vol. 147, no. 2, pp. 171-179, 2010.
82. A. Mehta, "Cystic fibrosis as a bowel cancer syndrome and the potential role of CK2,"Molecular and Cellular Biochemistry, vol. 316, no. 1-2, pp. 169-175, 2008.
83. H. Hamai, F. Keyserman, L. M. Quittell, and T. S. Worgall, "Defective CFTR increases synthesis and mass of sphingolipids that modulate membrane composition and lipid signaling," Journal of Lipid Research, vol. 50, no. 6, pp. 1101-1108, 2009.
84. M. Bodas, T. Min, S. Mazur, and N. Vij, "Critical modifier role of membrane-cystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema," Journal of Immunology, vol. 186, no. 1, pp. 602-613, 2011.
85. F. R. Long, R. S. Williams, and R. G. Castile, "Structural airway abnormalities in infants and young children with cystic fibrosis," The Journal of Pediatrics, vol. 144, no. 2, pp. 154-161, 2004.
86. S. P. De Langhe, G. Carraro, D. Warburton, M. K. Hajihosseini, and S. Bellusci, "Levels of mesenchymal FGFR2 signaling modulate smooth muscle progenitor cell commitment in the lung," Developmental Biology, vol. 299, no. 1, pp. 52-62, 2006.
87. A. Ornoy, J. Arnon, D. Katznelson, M. Granat, B. Caspi, and J. Chemke, "Pathological confirmation of cystic fibrosis in the fetus following prenatal diagnosis," American Journal ofMedical Genetics, vol. 28, no. 4, pp. 935-947, 1987.
88. L. C. Boujaoude, C. Bradshaw-Wilder, C. Mao et al., "Cystic fibrosis transmembrane regulator regulates uptake of sphingoid base phosphates and lysophosphatidic acid: modulation of cellular activity of sphingosine 1-phosphate," The Journal of Biological Chemistry, vol. 276, no. 38, pp. 35258-35264, 2001.
89. S. A. McColley, V. Stellmach, S. R. Boas, M. Jain, and S. E. Crawford, "Serum vascular endothelial growth factor is elevated in cystic fibrosis and decreases with treatment of acute pulmonary exacerbation," American Journal of Respiratory and Critical Care Medicine, vol. 161, no. 6, pp. 1877-1880, 2000.
90. M. B. Brown, W. R. Hunt, J. E. Noe et al., "Loss of cystic fibrosis transmembrane conductance regulator impairs lung endothelial cell barrier function and increases susceptibility to microvascular damage from cigarette smoke," Pulmonary Circulation, vol. 4, no. 2, pp. 260-268, 2014.
91. V. Teichgräber, M. Ulrich, N. Endlich et al., "Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis," Nature Medicine, vol. 14, no. 4, pp. 382-391, 2008.
92. A. Caretti, A. Bragonzi, M. Facchini et al., "Anti-inflammatory action of lipid nanocarrier-delivered myriocin: therapeutic potential in cystic fibrosis," Biochimica et Biophysica Acta: General Subjects, vol. 1840, no. 1, pp. 586-594, 2014.
93. S. Sahu andW. S. Lynn, "Lipid composition of airway secretions frompatients with asthma and patients with cystic fibrosis," The American Review of Respiratory Disease, vol. 115, no. 2, pp. 233-239, 1977.
94. S. Sahu and W. S. Lynn, "Lipid composition of sputum from patients with asthma and patients with cystic fibrosis," Inflammation, vol. 3, no. 1, pp. 27-36, 1978.
95. M. Brodlie, M. C. McKean, G. E. Johnson et al., "Ceramide is increased in the lower airway epithelium of people with advanced cystic fibrosis lung disease," American Journal of Respiratory and Critical Care Medicine, vol. 182, no. 3, pp. 369-375, 2010.
96. M.Ulrich,D.Worlitzsch, S.Viglio et al., "Alveolar inflammation in cystic fibrosis," Journal of Cystic Fibrosis, vol. 9, no. 3, pp. 217-227, 2010.
97. K. A. Becker, B. Henry, R. Ziobro, B. Tümmler, E. Gulbins, and H. Grassmé, "Role of CD95 in pulmonary inflammation and infection in cystic fibrosis," Journal of Molecular Medicine, vol. 90, no. 9, pp. 1011-1023, 2012.
98. C. Guilbault, G. Wojewodka, Z. Saeed et al., "Cystic fibrosis fatty acid imbalance is linked to ceramide deficiency and corrected by fenretinide," American Journal of Respiratory Cell and Molecular Biology, vol. 41, no. 1, pp. 100-106, 2009.
99. M.-C. Morales, G. Pérez-Yarza, N. N. Rementería et al., "4-HPR-mediated leukemia cell cytotoxicity is triggered by ceramide-induced mitochondrial oxidative stress and is regulated downstreamby Bcl-2," Free Radical Research, vol. 41, no. 5, pp. 591-601, 2007.
100. K. A. Becker, J. Riethmüller, A. Lüth, G. Döring, B. Kleuser, and E. Gulbins, "Acid sphingomyelinase inhibitors normalize pulmonary ceramide and inflammation in cystic fibrosis," American Journal of Respiratory Cell andMolecular Biology, vol. 42, no. 6, pp. 716-724, 2010.
101. L. Guillot, N. Nathan,O. Tabary et al., "Alveolar epithelial cells: master regulators of lung homeostasis," International Journal of Biochemistry and Cell Biology, vol. 45, no. 11, pp. 2568-2573, 2013.
102. V. Sender and C. Stamme, "Lung cell-specific modulation of LPS-induced TLR4 receptor and adaptor localization," Communicative and Integrative Biology, vol. 7, no. 5, Article ID e29053, 2014.
103. K. A. Becker, B. Tümmler, E. Gulbins, and H. Grassmé, "Accumulation of ceramide in the trachea and intestine of cystic fibrosis mice causes inflammation and cell death," Biochemical and Biophysical Research Communications, vol. 403, no. 3-4, pp. 368-374, 2010.
104. E. Barasch, Z. Vered, A. Shotan,D.Freimark, andB.Rabinowitz, "Dissecting aortic aneurysm-failure of standard noninvasive and invasive diagnostic techniques," Cardiology, vol. 79, no. 4, pp. 309-313, 1991.
105. A. Di, M. E. Brown, L. V. Deriy et al., "CFTR regulates phagosome acidification inmacrophages and alters bactericidal activity," Nature Cell Biology, vol. 8, no. 9, pp. 933-944, 2006.
106. Y. Zhang, X. Li, H. Grassmé, G. Döring, and E. Gulbins, "Alterations in ceramide concentration and pH determine the release of reactive oxygen species by Cftr-deficient macrophages on infection," The Journal of Immunology, vol. 184, no. 9, pp. 5104-5111, 2010.
107. J. Noe, D. Petrusca, N. Rush et al., "CFTR regulation of intracellular pH and ceramides is required for lung endothelial cell apoptosis," American Journal of Respiratory Cell and Molecular Biology, vol. 41, no. 3, pp. 314-323, 2009.
108. Y. Pewzner-Jung, S. Tavakoli Tabazavareh, H. Grassmé et al., "Sphingoid long chain bases prevent lung infection by Pseudomonas aeruginosa," EMBO Molecular Medicine, vol. 6, no. 9, pp. 1205-1214, 2014.
109. H. Barriere, M. Bagdany, F. Bossard et al., "Revisiting the role of cystic fibrosis transmembrane conductance regulator and counterion permeability in the pH regulation of endocytic organelles," Molecular Biology of the Cell, vol. 20, no. 13, pp. 3125-3141, 2009.
110. P. M. Haggie and A. S. Verkman, "Unimpaired lysosomal acidification in respiratory epithelial cells in cystic fibrosis," The Journal of Biological Chemistry, vol. 284, no. 12, pp. 7681-7686, 2009.
111. R. Ziobro, B. Henry, M. J. Edwards, A. B. Lentsch, and E. Gulbins, "Ceramide mediates lung fibrosis in cystic fibrosis," Biochemical and Biophysical Research Communications, vol. 434, no. 4, pp. 705-709, 2013.
112. D.-H. Kim, Y.-S. Lee, Y.-M. Lee, S. Oh, Y.-P. Yun, and H.-S. Yoo, "Elevation of sphingoid base 1-phosphate as a potential contributor to hepatotoxicity in fumonisin B1-exposed mice," Archives of Pharmacal Research, vol. 30,no. 8, pp. 962-969, 2007.
113. N. Sharma, Q. He, and R. P. Sharma, "Sphingosine kinase activity confers resistance to apoptosis by fumonisin B1 in human embryonic kidney (HEK-293) cells," Chemico-Biological Interactions, vol. 151, no. 1, pp. 33-42, 2004.
114. A. Rab, S. M. Rowe, S. V. Raju, Z. Bebok, S. Matalon, and J. F. Collawn, "Cigarette smoke and CFTR: implications in the pathogenesis of COPD," American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 305, no. 8, pp. L530-L541, 2013.
115. H.Grassmé, A.Carpinteiro, M. J. Edwards, E.Gulbins, andK.A. Becker, "Regulation of the inflammasome by ceramide in cystic fibrosis lungs," Cellular Physiology and Biochemistry, vol. 34, no. 1, pp. 45-55, 2014.
116. J. Riethmüller, J. Anthonysamy, E. Serra,M. Schwab, G.Döring, and E.Gulbins, "Therapeutic efficacy and safety of amitriptyline in patients with cystic fibrosis," Cellular Physiology and Biochemistry, vol. 24, no. 1-2, pp. 65-72, 2009.
117. L. Nährlich, J. G. Mainz, C. Adams et al., "Therapy of CF-patients with amitriptyline and placebo-a randomised, double-blind, placebo-controlled phase IIbmulticenter, cohortstudy," Cellular Physiology and Biochemistry, vol. 31, no. 4-5, pp. 505-512, 2013.
118. Y. Itokazu, R. E. Pagano, A. S. Schroeder, S. M. OGrady, A. H. Limper, and D. L. Marks, "Reduced GM1 ganglioside in CFTR-deficient human airway cells results in decreased β 1-integrin signaling and delayed wound repair," American Journal of Physiology: Cell Physiology, vol. 306, no. 9, pp. C819-C830, 2014.
119. S. Wang, P. Robinet, J. D. Smith, and K. Gulshan, "Freecholesterol-mediated autophagy of ORMDL1 stimulates sphingomyelin biosynthesis," Autophagy, vol. 11, no. 7, pp. 1207-1208, 2015.
120. N. M.White, D. Jiang, J. D. Burgess, I. R. Bederman, S. F. Previs, and T. J. Kelley, "Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis," The American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 292, no. 2, pp. L476-L486, 2007.
121. D. M. Mannino, D. M. Homa, L. J. Akinbami, E. S. Ford, and S. C. Redd, "Chronic obstructive pulmonary disease surveillance-United States, 1971-2000," Morbidity and Mortality Weekly Report. Surveillance Summaries, vol. 51, no. 6, pp. 1-16, 2002.
122. A. S. Gershon, L. Warner, P. Cascagnette, J. C. Victor, and T. To, "Lifetime risk of developing chronic obstructive pulmonary disease: a longitudinal population study," The Lancet, vol. 378, no. 9795, pp. 991-996, 2011.
123. S. S. Salvi and P. J. Barnes, "Chronic obstructive pulmonary disease in non-smokers," The Lancet, vol. 374, no. 9691, pp. 733-743, 2009.
124. J. E. McDonough, R. Yuan, M. Suzuki et al., "Small-airway obstruction and emphysema in chronic obstructive pulmonary disease," The New England Journal of Medicine, vol. 365, no. 17, pp. 1567-1575, 2011.
125. Y. S. Prakash, "Airway smooth muscle in airway reactivity and remodeling: what have we learned?" American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 305, no. 12, pp. L912-L933, 2013.
126. C. R. Laratta and S. van Eeden, "Acute exacerbation of chronic obstructive pulmonary disease: cardiovascular links," BioMed Research International, vol. 2014, Article ID 528789, 18 pages, 2014.
127. R. Aldonyte, E. Bagdonas, J. Raudoniute, and I. Bruzauskaite, "Novel aspects of pathogenesis and regeneration mechanisms in COPD," International Journal of ChronicObstructive Pulmonary Disease, vol. 10, no. 1, pp. 995-1013, 2015.
128. C. Martin, J. Frija-Masson, and P.-R. Burgel, "Targetingmucus hypersecretion: new therapeutic opportunities for COPD?" Drugs, vol. 74, no. 10, pp. 1073-1089, 2014.
129. T. A. Packard, Q. Z. Li, G. P. Cosgrove, R. P. Bowler, and J. C. Cambier, "COPD is associatedwith production of autoantibodies to a broad spectrumof self-antigens, correlative with disease phenotype," Immunologic Research, vol. 55, no. 1-3, pp. 48-57, 2013.
130. S. Fujii, H. Hara, J. Araya et al., "Insufficient autophagy promotes bronchial epithelial cell senescence in chronic obstructive pulmonary disease,"OncoImmunology, vol. 1, no. 5,pp. 630-641, 2012.
131. S.W.Ryter, S.-J. Lee, andA. M. K. Choi, "Autophagy in cigarette smoke-induced chronic obstructive pulmonary disease," Expert Review of Respiratory Medicine, vol. 4, no. 5, pp. 573-584, 2010.
132. I. Petrache, T. R. Medler, A. T. Richter et al., "Superoxide dismutase protects against apoptosis and alveolar enlargement induced by ceramide," American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 295, no. 1, pp. L44-L53, 2008.
133. M. Levy, E. Khan, M. Careaga, and T. Goldkorn, "Neutral sphingomyelinase 2 is activated by cigarette smoke to augment ceramide-induced apoptosis in lung cell death," American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 297, no. 1, pp. L125-L133, 2009.
134. T. Goldkorn, S. Filosto, and S. Chung, "Lung injury and lung cancer caused by cigarette smoke-induced oxidative stress: molecularmechanisms and therapeutic opportunities involving the ceramide-generating machinery and epidermal growth factor receptor," Antioxidants and Redox Signaling, vol. 21, no. 15, pp. 2149-2174, 2014.
135. S. Chung, S. Vu, S. Filosto, and T. Goldkorn, "Src regulates cigarette smoke-induced ceramide generation via neutral sphingomyelinase 2 in the airway epithelium," American Journal of Respiratory Cell and Molecular Biology, vol. 52, no. 6, pp. 738-748, 2015.
136. S. Sammani, L. Moreno-Vinasco, T. Mirzapoiazova et al., "Differential effects of sphingosine 1-phosphate receptors on airway and vascular barrier function in the murine lung," American Journal of Respiratory Cell and Molecular Biology, vol. 43, no. 4, pp. 394-402, 2010.
137. Y. Gon, M. R. Wood, W. B. Kiosses et al., "S1P3 receptor-induced reorganization of epithelial tight junctions compromises lung barrier integrity and is potentiated by TNF," Proceedings of the National Academy of Sciences of the United States of America, vol. 102, no. 26, pp. 9270-9275, 2005.
138. F. Cordts, S. Pitson, C. Tabeling et al., "Expression profile of the sphingosine kinase signalling system in the lung of patientswith chronic obstructive pulmonary disease," Life Sciences, vol. 89, no. 21-22, pp. 806-811, 2011.
139. E.-J. D. Oudijk, J.-W. J. Lammers, and L. Koenderman, "Systemic inflammation in chronic obstructive pulmonary disease," The European Respiratory Journal. Supplement, vol. 22, no. 46, pp. 5s-13s, 2003.
140. R. Aldonyte, S. Eriksson, E. Piitulainen, A. Wallmark, and S. Janciauskiene, "Analysis of systemic biomarkers in COPD patients," COPD: Journal of Chronic Obstructive Pulmonary Disease, vol. 1, no. 2, pp. 155-164, 2004.
141. T. R. Martin, G. Raghu, R. J. Maunder, and S. C. Springmeyer, "The effects of chronic bronchitis and chronic air-flow obstruction on lung cell populations recovered by bronchoalveolar lavage," The American Review of Respiratory Disease, vol. 132, no. 2, pp. 254-260, 1985.
142. A. K. Ravi, S. Khurana, J. Lemon et al., "Increased levels of soluble interleukin-6 receptor and CCL3 in COPD sputum," Respiratory Research, vol. 15, article 103, 2014.
143. T. M. Bahr, G. J. Hughes, M. Armstrong et al., "Peripheral blood mononuclear cell gene expression in chronic obstructive pulmonary disease," American Journal of Respiratory Cell and Molecular Biology, vol. 49, no. 2, pp. 316-323, 2013.
144. E. D. Telenga, R. F. Hoffmann Ruben tKindt et al., "Untargeted lipidomic analysis in chronic obstructive pulmonary disease. Uncovering sphingolipids," American Journal of Respiratory and Critical Care Medicine, vol. 190, no. 2, pp. 155-164, 2014.
145. M. Tani, Y. Igarashi, and M. Ito, "Involvement of neutral ceramidase in ceramide metabolism at the plasma membrane and in extracellular milieu,"The Journal of Biological Chemistry, vol. 280, no. 44, pp. 36592-36600, 2005.
146. R. P. Bowler, S. Jacobson, C. Cruickshank et al., "Plasma sphingolipids associated with chronic obstructive pulmonary disease phenotypes," American Journal of Respiratory and Critical Care Medicine, vol. 191, no. 3, pp. 275-284, 2015.
147. J.D. Brain, "Macrophage damage in relation to the pathogenesis of lung diseases," Environmental Health Perspectives, vol. 35, pp. 21-28, 1980.
148. P. Kirkham, "Oxidative stress and macrophage function: a failure to resolve the inflammatory response," Biochemical Society Transactions, vol. 35, no. 2, pp. 284-287, 2007.
149. D. N. Petrusca, Y. Gu, J. J. Adamowicz et al., "Sphingolipidmediated inhibition of apoptotic cell clearance by alveolar macrophages," The Journal of Biological Chemistry, vol. 285, no. 51, pp. 40322-40332, 2010.
150. D. Zabini, S. Crnkovic, H. Xu et al., "High-mobility group box-1 induces vascular remodelling processes via c-Jun activation," Journal of Cellular and Molecular Medicine, vol. 19, no. 5, pp. 1151-1161, 2015.
151. E. Stacher, B. B. Graham, J.M. Hunt et al., "Modern age pathology of pulmonary arterial hypertension," American Journal of Respiratory and Critical Care Medicine, vol. 186, no. 3, pp. 261-272, 2012.
152. M. Bodas, T. Min, and N. Vij, "Lactosylceramide-accumulation in lipid-rafts mediate aberrant-autophagy, inflammation and apoptosis in cigarette smoke induced emphysema," Apoptosis, vol. 20, pp. 725-739, 2015.