CFTR activity and mitochondrial function

Redox Biology

Volumn 1, Issue 1, 2013, Pages 190-202

  Valdivieso, Angel Gabriel ^a   Santa Coloma, Tomás A ^a,b  

^a PONTIFICIA UNIVERSIDAD CATÓLICA ARGENTINA   (Argentina)

^b Facultad de Ciencias Naturales e Instituto M Lillo   (Argentina)

Author keywords

Apoptosis; CFTR; Cystic Fibrosis; Innate immunity; Mitochondrial complex I; Reactive oxygen species

Indexed keywords

CALCIUM; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; GLUTATHIONE; MEMBRANE PROTEIN; MESSENGER RNA; PEROXIREDOXIN; PIOGLITAZONE; PROTEIN CISD1; REACTIVE OXYGEN METABOLITE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); SUPEROXIDE DISMUTASE; UNCLASSIFIED DRUG; CALCIUM ION;

APOPTOSIS; AUTOPHAGY; CALCIUM HOMEOSTASIS; CISD1 GENE; CRYSTAL STRUCTURE; CYSTIC FIBROSIS; DIABETES MELLITUS; DISORDERS OF MITOCHONDRIAL FUNCTIONS; DRUG RECEPTOR BINDING; GENE; GENE EXPRESSION; HUMAN; INNATE IMMUNITY; MITOCHONDRIAL RESPIRATION; MT DN4 GENE; NONHUMAN; NUCLEOTIDE SEQUENCE; OXIDATIVE PHOSPHORYLATION; OXIDATIVE STRESS; PATHOPHYSIOLOGY; PHENOTYPE; PNEUMONIA; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN FUNCTION; REVIEW; CFTR GENE; CHRONIC INFLAMMATION; CORRELATIONAL STUDY; DN4 GENE; GENE FUNCTION; MITOCHONDRIAL GENE; ANIMAL; METABOLISM; MITOCHONDRION;

ANIMALS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; HUMANS; MITOCHONDRIA;

EID: 84878874710     PISSN: 22132317     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.redox.2012.11.007     Document Type: Review

References (307)

1. P.M. Quinton, Cystic fibrosis: lessons from the sweat gland, Physiology (Bethesda) 22 (2007) 212-225.
2. J. Zielenski, Genotype and phenotype in cystic fibrosis, Respiration 67
3. H. Heijerman, Infection and inflammation in cystic fibrosis: a short review,Journal of Cystic Fibrosis 4 (Suppl. 2) (2005) 3-5.
4. C.U. Lowe, CD. May, S.C. Reed, Fibrosis of the pancreas in infants and children; a statistical study of clinical and hereditary features, American Journal of Diseases of Children 78 (1949) 349-374.
5. CD. May, C.U. Lowe, Fibrosis of the pancreas in infants and children; an illustrated review of certain clinical features with special emphasis on the pulmonary and cardiac aspects, Journal of Pediatrics 34 (1949) 663-687.
6. J.R. Riordan, J.M. Rommens, B. Kerem, N. Alon, R. Rozmahel, Z. Grzelczak, J. Zielenski, S. Lok, N. Plavsic, J.L. Chou, M.L. Drumm, M.C Iannuzzi, F.S. Collins, L.C. Tsui, Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA, Science 245 (1989) 1066-1073.
7. L.C. Tsui, J.M. Rommens, J. Burns, S. Zengerling, J.R. Riordan, L.R. Carlock, K.H. Grzeschik, M. Buchwald, Progress towards cloning the cystic fibrosis gene, Philosophical Transactions of the ROYAL Society of London Series B, Biological Sciences 319 (1988) 263-273.
8. J.M. Rommens, M.C. Iannuzzi, B. Kerem, M.L. Drumm, G. Melmer, M. Dean, R. Rozmahel, J.L. Cole, D. Kennedy, N. Hidaka, M. Zsiga, M. Buchwald, J.R. Riordan, L.C. Tsui, F.S. Collins, Identification of the cystic fibrosis gene: chromosome walking and jumping, Science 245 (1989) 1059-1065.
9. M.L. Drumm, H.A. Pope, W.H. Cliff, J.M. Rommens, S.A. Marvin, LC Tsui, F.S. Collins, R.A. Frizzell, J.M. Wilson, Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer, Cell 62 (1990) 1227-1233.
10. R.J. Gregory, S.H. Cheng, D.P. Rich, J. Marshall, S. Paul, K. Hehir, L. Ostedgaard, K.W. Klinger, M.J. Welsh, A.E. Smith, Expression and characterization of the cystic fibrosis transmembrane conductance regulator, Nature 347 (1990) 382-386.
11. D.M. Jefferson, J.D. Valentich, F.C Marini, S.A. Grubman, M.C. Iannuzzi, H.L. Dorkin, M. Li, K.W. Klinger, M.J. Welsh, Expression of normal and cystic fibrosis phenotypes by continuous airway epithelial cell lines, American Journal of Physiology 259 (1990) L496-505.
12. D.P. Rich, M.P. Anderson, RJ. Gregory, S.H. Cheng, S. Paul, D.M. Jefferson, J.D. McCann, K.W. Klinger, A.E. Smith, M.J. Welsh, Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells, Nature 347 (1990)358-363.
13. M.P. Anderson, RJ. Gregory, S. Thompson, D.W. Souza, S. Paul, R.C. Mulligan, A.E. Smith, M.J. Welsh, Demonstration that CFTR is a chloride channel by alteration of its anion selectivity, Science 253 (1991) 202-205.
14. M.P. Anderson, D.P. Rich, RJ. Gregory, A.E. Smith, M.J. Welsh, Generation of cAMP-activated chloride currents by expression of CFTR, Science 251 (1991) 679-682.
15. CE. Bear, F. Duguay, A.L. Naismith, N. Kartner, J.W. Hanrahan, J.R. Riordan, Cl~ channel activity in Xenopus oocytes expressing the cystic fibrosis gene, Journal of Biological Chemistry 266 (1991) 19142-19145.
16. D.N. Sheppard, M.J. Welsh, Structure and function of the CFTR chloride channel, Physiological Reviews 79 (1999) S23-45.
17. N. Gahm, H. Shwachman, Studies in cystic fibrosis of the pancreas; a simple test for the detection of excessive chloride on the skin, New England Journal of Medicine 255 (1956) 999-1001.
18. GJ. Barbero, M.S. Sibinga, The electrolyte abnormality in cystic fibrosis, Pediatric Clinics of North America 11 (1964) 983-1001.
19. L. Hansen, M. Buechele, J. Koroshec, WJ. Warwick, Sweat chloride assay for cystic fibrosis. Using pilocarpine iontophoresis stimulation, filter paper collection and cotlove chloridometer analysis, Minnesota Medicine 50 (1967)1191-1196.
20. M.L. McCombs, Research in cystic fibrosis: a review, Texas Reports on Biology and Medicine 31 (1973) 615-629.
21. J. Steinrud, S. Winkel, E.W. Flensborg, Screening for cystic fibrosis with chloride electrode. An investigation of sweat chloride with chloride electrode orion 417 in normal persons and in patients with cystic fibrosis, Danish Medical Bulletin 21 (1974) 251-255.
22. B.H. Bowman, D.R. Barnett, Recent advances in cystic fibrosis research, Birth Defects Original Article Series 12 (1976) 197-212.
23. P.M. Quinton, Chloride impermeability in cystic fibrosis, Nature 301 (1983) 421-422.
24. R.C. Boucher, D.W. Ross, M.R. Knowles, J.T. Gatzy, J.C Parker, Cl~ permeabilities in red blood cells and peripheral blood lymphocytes from cystic fibrosis and control subjects, Pediatric Research 18 (1984) 1336-1339.
25. J.H. Widdicombe, M.J. Welsh, W.E. Finkbeiner, Cystic fibrosis decreases the apical membrane chloride permeability of monolayers cultured from cells of tracheal epithelium, Proceedings of the National Academy of Sciences of the United States of America 82 (1985) 6167-6171.
26. P.M. Quinton, Missing Cl~ conductance in cystic fibrosis, American Journal of Physiology 251 (1986) C649-652.
27. N.P. Illsley, A.S. Verkman, Membrane chloride transport measured using a chloride-sensitive fluorescent probe, Biochemistry 26 (1987) 1215-1219.
28. J.H. Widdicombe, Altered chloride transport of tracheal epithelium in cystic fibrosis, Progress in Clinical and Biological Research 254 (1987) 115-126.
29. H.M. Berschneider, M.R. Knowles, R.G. Azizkhan, R.C. Boucher, N.A. Tobey, R.C. Orlando, D.W. Powell, Altered intestinal chloride transport in cystic fibrosis, FASEB Journal 2 (1988) 2625-2629.
30. R.C. Boucher, C.U. Cotton, J.T. Gatzy, M.R. Knowles, J.R. Yankaskas, Evidence for reduced Cl~ and increased Na+ permeability in cystic fibrosis human primary cell cultures, Journal of Physiology 405 (1988) 77-103.
31. J.H. Chen, H. Schulman, P. Gardner, A cAMP-regulated chloride channel in lymphocytes that is affected in cystic fibrosis, Science 243 (1989) 657-660.
32. C.A. Boyd, Function of cystic fibrosis gene product, Lancet 336 (1990) 938.
33. R. Busch, On the history of cystic fibrosis, Nordisk Medicinhistorisk Arsbok (1991)95-98.
34. P.M. Quinton, Physiological basis of cystic fibrosis: a historical perspective, Physiological Reviews 79 (1999) S3-S22.
35. E. Kerem, M. Corey, B.S. Kerem, J. Rommens, D. Markiewicz, H. Levison, L.C. Tsui, P. Durie, The relation between genotype and phenotype in cystic fibrosis-analysis of the most common mutation (delta F508), New England Journal of Medicine 323 (1990) 1517-1522.
36. E. Mateu, F. Calafell, M.D. Ramos, T. Casals, J. Bertranpetit, Can a place of origin of the main cystic fibrosis mutations be identified? American Journal of Human Genetics 70 (2002) 257-264.
37. P.G. Noone, M.R. Knowles, 'CFTR-opathies': disease phenotypes associated with cystic fibrosis transmembrane regulator gene mutations, Respiratory Research 2 (2001) 328-332.
38. T. Lange, P. Jungmann, J. Haberle, S. Falk, A. Duebbers, R. Bruns, A. Ebner, P. Hinterdorfer, H. Oberleithner, H. Schillers, Reduced number of CFTR molecules in erythrocyte plasma membrane of cystic fibrosis patients, Molecular Membrane Biology 23 (2006) 317-323.
39. A.E. Mulberg, E.B. Wiedner, X. Bao, J. Marshall, D.M. Jefferson, S.M. Altschuler, Cystic fibrosis transmembrane conductance regulator protein expression in brain, Neuroreport 5 (1994) 1684-1688.
40. P.C. Levesque, PJ. Hart, J.R. Hume, J.L. Kenyon, B. Horowitz, Expression of cystic fibrosis transmembrane regulator Cl~ channels in heart, Circulation Research 71 (1992) 1002-1007.
41. B. Horowitz, S.S. Tsung, P. Hart, P.C. Levesque, J.R. Hume, Alternative splicing of CFTR Cl~ channels in heart, American Journal of Physiology 264 (1993) H2214-2220.
42. E.F. Tizzano, D. Chitayat, M. Buchwald, Cell-specific localization of CFTR mRNA shows developmentally regulated expression in human fetal tissues, Human Molecular Genetics 2 (1993) 219-224.
43. H.C. Chan, 0_.X. Shi, C.X. Zhou, X.F. Wang, W.M. Xu, W.Y. Chen, A.J. Chen, Y. Ni, Y.Y. Yuan, Critical role of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm, Molecular and Cellular Endocrinology 250 (2006)106-113.
44. I. Kogan, M. Ramjeesingh, C. Li, J.F. Kidd, Y. Wang, E.M. Leslie, S.P. Cole, C.E. Bear, CFTR directly mediates nucleotide-regulated glutathione flux, EMBO Journal 22 (2003) 1981-1989.
45. I.L. Reisin, A.G. Prat, E.H. Abraham, J.F. Amara, R.J. Gregory, D.A. Ausiello, H.F. Cantiello, The cystic fibrosis transmembrane conductance regulator is a dual ATP and chloride channel, Journal of Biological Chemistry 269 (1994) 20584-20591.
46. C. Li, M. Ramjeesingh, C.E. Bear, Purified cystic fibrosis transmembrane conductance regulator (CFTR) does not function as an ATP channel, Journal of Biological Chemistry 271 (1996) 11623-11626.
47. A.G. Prat, I.L. Reisin, D.A. Ausiello, H.F. Cantiello, Cellular ATP release by the cystic fibrosis transmembrane conductance regulator, American Journal of Physiology 270 (1996) C538-545.
48. H.F. Cantiello, Nucleotide transport through the cystic fibrosis transmembrane conductance regulator, Bioscience Reports 17 (1997) 147-171.
49. S. Devidas, W.B. Guggino, The cystic fibrosis transmembrane conductance regulator and ATP, Current Opinion in Cell Biology 9 (1997) 547-552.
50. E.A. Pasyk, J.K. Foskett, Cystic fibrosis transmembrane conductance regulator-associated ATP and adenosine 3'-phosphate 5'-phosphosulfate channels in endoplasmic reticulum and plasma membranes, Journal of Biological Chemistry 272 (1997) 7746-7751.
51. H.F. Cantiello, G.R. Jackson Jr., C.F. Grosman, A.G. Prat, S.C. Borkan, Y. Wang, I.L. Reisin, C.R. O'Riordan, D.A. Ausiello, ATP Electro diffusional, movement through the cystic fibrosis transmembrane conductance regulator, American journal of physiology 274 (1998) C799-809.
52. E.H. Abraham, K.M. Sterling, RJ. Kim, A.Y. Salikhova, H.B. Huffman, M.A. Crockett, N. Johnston, H.W. Parker, W.E. Boyle Jr., A. Hartov, E. Demidenko, J. Efird, J. Kahn, S.A. Grubman, D.M. Jefferson, S.C. Robson, J.H. Thakar, A. Lorico, G. Rappa, A.C. Sartorelli, P. Okunieff, Erythrocyte membrane ATP binding cassette (ABC) proteins: MRP1 and CFTR as well as CD39 (ecto-apyrase) involved in RBC ATP transport and elevated blood plasma ATP of cystic fibrosis, Blood cells, Molecules and Diseases 27 (2001) 165-180.
53. H.F. Cantiello, ATP Electrodiffusional, movement through CFTR and other ABC transporters, Pflugers Archiv 443 (2001) S22-27.
54. D. Reigada, C.H. Mitchell, Release of ATP from retinal pigment epithelial cells involves both CFTR and vesicular transport, American Journal of Physiology Cell Physiology 288 (2005) C132-140.
55. E.M. Schwiebert, D.J. Benos, M.E. Egan, M.J. Stutts, W.B. Guggino, CFTR is a conductance regulator as well as a chloride channel, Physiological Reviews 79 (1999)S145-166.
56. R. Alzamora, J.D. King Jr., K.R. Hallows, CFTR regulation by phosphorylation, Methods in Molecular Biology 741 (2011) 471-488.
57. H. Fischer, T.E. Machen, The tyrosine kinase p60c-src regulates the fast gate of the cystic fibrosis transmembrane conductance regulator chloride channel, Biophysical Journal 71 (1996) 3073-3082.
58. B.J. Allan, K.T. Izutsu, B.W. Ramsey, M.M. Schubert, W.Y. Ensign, E.L. Truelove, Cyclic nucleotide responses in control and cystic fibrosis labial glands, American Journal of Physiology 258 (1990) R1320-1326.
59. D. Faria, R. Schreiber, K. Kunzelmann, CFTR is activated through stimulation of purinergic P2Y2 receptors, Pflugers Archiv 457 (2009) 1373-1380.
60. C. Li, A.P. Naren, Analysis of CFTR interactome in the macromolecular complexes, Methods in Molecular Biology 741 (2011) 255-270.
61. A.P. Naren, B. Cobb, C. Li, K. Roy, D. Nelson, G.D. Heda, J. Liao, K.L. Kirk, E.J. Sorscher, J. Hanrahan, J.P. Clancy, A macromolecular complex of beta 2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phospho-protein 50 is regulated by PKA, Proceedings of the National Academy of Sciences of the United States of America 100 (2003) 342-346.
62. W. Zhang, H. Penmatsa, A. Ren, C. Punchihewa, A. Lemoff, B. Yan, N. Fujii, A.P. Naren, Functional regulation of cystic fibrosis transmembrane conductance regulator-containing macromolecular complexes: a small-molecule inhibitor approach, Biochemical Journal 435 (2011) 451-462.
63. C. Li, A.P. Naren, Macromolecular complexes of cystic fibrosis transmem-brane conductance regulator and its interacting partners, Pharmacology and Therapeutics 108 (2005) 208-223.
64. M. Buchwald, R. Sood, W. Auerbach, Regulation of expression of CFTR in human intestinal epithelial cells, Advances in Experimental Medicine and Biology 290 (1991) 241-250. (discussion 242-250).
65. W. Breuer, N. Kartner, J.R. Riordan, Z.I. Cabantchik, Induction of expression of the cystic fibrosis transmembrane conductance regulator, Journal of Biological Chemistry 267 (1992) 10465-10469.
66. R.A. McDonald, R.P. Matthews, R.L. Idzerda, G.S. McKnight, Basal expression of the cystic fibrosis transmembrane conductance regulator gene is dependent on protein kinase A activity, Proceedings of the National Academy of Sciences of the United States of America 92 (1995) 7560-7564.
67. F. Besancon, G. Przewlocki, I. Baro, A.S. Hongre, D. Escande, A. Edelman, Interferon-gamma downregulates CFTR gene expression in epithelial cells, American Journal of Physiology 267 (1994) C1398-1404.
68. A. Mularoni, L. Beck, R. Sadir, G.L. Adessi, M. Nicollier, Down-regulation by progesterone of CFTR expression in endometrial epithelial cells: a study by competitive RT-PCR, Biochemical and Biophysical Research Communications 217 (1995) 1105-1111.
69. E.G. Cafferata, A.M. González-Guerrico, L. Giordano, O.H. Pivetta, T.A. Santa-Coloma, Interleukin-1beta regulates CFTR expression in human intestinal T84 cells, Biochimica et Biophysica Acta 1500 (2000) 241-248.
70. E.G. Cafferata, A.M. González-Guerrico, O.H. Pivetta, T.A. Santa-Coloma, NF-kappaB activation is involved in regulation of cystic fibrosis transmembrane conductance regulator (CFTR) by interleukin-1beta, Journal of Biological Chemistry 276 (2001) 15441-15444.
71. E.G.A. Cafferata, Regulación del gen CFTR (afectado en fibrosis quística) en células de carcinoma de colon humano T84, Thesis dissertation School of Pharmacy and Biochemistry, University of Buenos Aires, (2002) 1-106.
72. E.Osika, J.M.Cavaillon, K.Chadelat, M.Boule, C.Fitting, G.Tournier, A. Clement, Distinct sputum cytokine profiles in cystic fibrosis and other chronic inflammatory airway disease, European Respiratory Journal 14 (1999) 339-346.
73. L. Rochwerger, M. Buchwald, Stimulation of the cystic fibrosis transmem-brane regulator expression by estrogen in vivo, Endocrinology 133 (1993) 921-930.
74. D.K. Rowlands, L.L. Tsang, Y.G. Cui, Y.W. Chung, L.N. Chan, C.Q. Liu, T. James, H.C. Chan, Upregulation of cystic fibrosis transmembrane conductance regulator expression by oestrogen and Bak Foong Pill in mouse uteri, Cell Biology International 25 (2001) 1033-1035.
75. M. Longo, M. Brama, M. Marino, S. Bernardini, K.S. Korach, W.C. Wetsel, R. Scandurra, T. Faraggiana, G. Spera, R. Baron, A. Teti, S. Migliaccio, Interaction of estrogen receptor alpha with protein kinase C alpha and c-Src in osteoblasts during differentiation, Bone 34 (2004) 100-111.
76. S.H. Chotirmall, C.M. Greene, I.K. Oglesby, W. Thomas, S.J. O'Neill, B.J. Harvey, N.G. McElvaney, 17Beta-estradiol inhibits IL-8 in cystic fibrosis by up-regulating secretory leucoprotease inhibitor, American Journal of Respiratory and Critical Care Medicine 182 (2010) 62-72.
77. Y.J. Dong, A.C. Chao, K. Kouyama, Y.P. Hsu, R.C. Bocian, R.B. Moss, P. Gardner, Activation of CFTR chloride current by nitric oxide in human T lymphocytes, EMBO Journal 14 (1995) 2700-2707.
78. T. Jilling, I.Y. Haddad, S.H. Cheng, S. Matalon, Nitric oxide inhibits hetero-logous CFTR expression in polarized epithelial cells, American Journal of Physiology 277 (1999) L89-96.
79. J. Texereau, S. Marullo, D. Hubert, J. Coste, D.J. Dusser, J. Dall'Ava-Santucci, A.T. Dinh-Xuan, Nitric oxide synthase 1 as a potential modifier gene of decline in lung function in patients with cystic fibrosis, Thorax 59 (2004) 156-158.
80. A.C. Skinn, W.K. MacNaughton, Nitric oxide inhibits cAMP-dependent CFTR trafficking in intestinal epithelial cells, American Journal of Physiology Gastrointestinal and Liver Physiology 289 (2005) G739-744.
81. M. Baudouin-Legros, F. Brouillard, M. Cougnon, D. Tondelier, T. Leclerc, A. Edelman, Modulation of CFTR gene expression in HT-29 cells by extracellular hyperosmolarity, American Journal of Physiology-Cell Physiology 278 (2000) C49-56.
82. F. Qu, H.J. Liu, Y. Xiang, Y.R. Tan, C. Liu, X.L. Zhu, X.Q. Qin, Activation of CFTR trafficking and gating by vasoactive intestinal peptide in human bronchial epithelial cells, Journal of Cellular Biochemistry 112 (2011) 902-908.
83. N. Alcolado, D.J. Conrad, S. Rafferty, F.G. Chappe, V.M. Chappe, VIP-dependent increase in F508del-CFTR membrane localization is mediated by PKCepsilon, American Journal of Physiology-Cell Physiology 301 (2011) C53-65.
84. A.I. Mendes, P. Matos, S. Moniz, S. Luz, M.D. Amaral, C.M. Farinha, P. Jordan, Antagonistic regulation of cystic fibrosis transmembrane conductance regulator cell surface expression by protein kinases WNK4 and spleen tyrosine kinase, Molecular and Cellular Biology 31 (2011) 4076-4086.
85. S. Luz, P. Kongsuphol, A.I. Mendes, F. Romeiras, M. Sousa, R. Schreiber, P. Matos, P. Jordan, A. Mehta, M.D. Amaral, K. Kunzelmann, C.M. Farinha, Contribution of casein kinase 2 and spleen tyrosine kinase to CFTR trafficking and protein kinase A-induced activity, Molecular and Cellular Biology 31 (2011) 4392-4404.
86. A.M. Gonzalez-Guerrico, E.G. Cafferata, M. Radrizzani, F. Marcucci, D. Gruenert, O.H. Pivetta, R.R. Favaloro, R. Laguens, S.V. Perrone, G.C. Gallo, T.A. Santa-Coloma, Tyrosine kinase c-Src constitutes a bridge between cystic fibrosis transmembrane regulator channel failure and MUC1 overexpression in cystic fibrosis, Journal of Biological Chemistry 277 (2002) 17239-17247.
87. A.G., Valdivieso, Modulación de la expresión del gen MTND4 mitocondrial mediada por la actividad del CFTR, Thesis dissertation, School of Natural and Exact Sciences, University of Buenos Aires, 2009, p. 1-118.
88. G.L. Taminelli, V. Sotomayor, A.G. Valdivieso, M.L. Teiber, M.C. Marín, T.A. Santa-Coloma, CISD1 codifies a mitochondrial protein upregulated by the CFTR channel, Biochemical and Biophysical Research Communications 365 (2008) 856-862.
89. K. Estell, G. Braunstein, T. Tucker, K. Varga, J.F. Collawn, L.M. Schwiebert, Plasma membrane CFTR regulates RANTES expression via its C-terminal PDZ-interacting motif, Molecular and Cellular Biology 23 (2003) 594-606.
90. M. Srivastava, O. Eidelman, H.B. Pollard, Pharmacogenomics of the cystic fibrosis transmembrane conductance regulator (CFTR) and the cystic fibrosis drug CPX using genome microarray analysis, Molecular Medicine 5 (1999) 753-767.
91. M. Srivastava, O. Eidelman, H.B. Pollard, cDNA microarrays for pharmaco-genomic analysis of cystic fibrosis, Methods in Molecular Medicine 70 (2002) 21-29.
92. P. Galvin, L.A. Clarke, S. Harvey, M.D. Amaral, Microarray analysis in cystic fibrosis, Journal of Cystic Fibrosis 3 (Suppl 2) (2004) 29-33.
93. M. Srivastava, O. Eidelman, C. Jozwik, C. Paweletz, W. Huang, P.L. Zeitlin, H.B. Pollard, Serum proteomic signature for cystic fibrosis using an antibody microarray platform, Molecular Genetics and Metabolism 87 (2006) 303-310.
94. A.G. Valdivieso, M. Clauzure, M.C. Marín, G.L. Taminelli, M.M. Massip Copiz,F. Sánchez, G. Schulman, M.L. Teiber, T.A. Santa-Coloma, The mitochondrial complex I activity is reduced in cells with impaired Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) function, PLoS ONE 7 (11) (2012) e48059, http://dx.doi.org/10.1371/journal.pone.0048059.
95. M. Clauzure, F. Sanchez, A.G. Valdivieso, M.M. Massip Copiz, G.L. Taminelli,G. Schulman, M.L. Teiber, T.A. Santa-Coloma, Abstract 596. Regulación de la actividad mitocondrial en Fibrosis Quística. Abstracts of the 56th annual meeting of the Argentine society for clinical investigation, Mar del Plata, Buenos Aires, Argentina 16-19 November 2011. Medicina (Buenos Aires) 71 (Suppl. III) (2012) 236.
96. M.L. Teiber, A.G. Valdivieso, M.M. Massip Copiz, M. Clauzure, F. Sánchez, G. Schulman, G.L. Taminelli, E.S. Pagano, T.A. Santa-Coloma, Abstract 188. La inhibición de la actividad o expresión del canal CFTR disminuye el potencial de membrana mitocondrial e induce apoptosis. Abstracts of the 55th annual meeting of the Argentine society for clinical research. Mar del Plata, Buenos Aires, Argentina; 17-20 November 2010, Medicina (Buenos Aires) 70 (Suppl. II) (2010) 109.
97. A.G. Valdivieso, G.L. Taminelli, M.C. Marín, E.S. Pagano, M.L. Teiber, T.A. Santa-Coloma, Abstract 529. La inhibición del transporte de Cl- a través del canal CFTR modula la actividad del complejo I mitocondrial. Abstracts of the 53th annual meeting of the Argentine society for clinical investigation. Mar del Plata, Buenos Aires, Argentina; 19-22 November 2008, Medicina (Buenos Aires) 68 (Suppl. II) (2008) 209.
98. G.L. Taminelli, A.G. Valdivieso, M.C. Marín, M.L. Teiber, E.S. Pagano, T.A. Santa-Coloma, Abstract 537. Caracterización parcial de una proteína nuclear de localización mitocondrial denominada CISD1 cuya expresión es modulada por el canal de Cl- CFTR. Abstracts of the 53th annual meeting of the Argentine society for clinical investigation. Mar del Plata, Buenos Aires, Argentina; 19-22 November 2008, Medicina (Buenos Aires) 68 (Suppl. II) (2008) 211.
99. A.G. Valdivieso, G.L. Taminelli, T.A. Santa-Coloma, Abstract 621. La Falla del CFTR en fibrosis quística reduce la actividad del complejo I mitocondrial. Abstracts of the 52th annual meeting of the Argentine society for clinical investigation. Mar del Plata, Buenos Aires, Argentina; 21-24 November 2007, Medicina (Buenos Aires) 67 (Suppl. III) (2007) 240.
100. A.G. Valdivieso, F. Marcucci, G.L. Taminelli, A.M. González-Guerrico, S. Alvarez, M.L. Teiber, M.A. Dankert, T.A. Santa-Coloma, The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis, Biochemical and Biophysical Research Communications 356 (2007) 805-809.
101. G.L. Taminelli, A.G. Valdivieso, T.A. Santa-Coloma, Abstract 625. Expresió n diferencial en fibrosis quística de una nueva proteína nuclear de localiza-ción mitocondrial denominada CISD1. Abstracts of the 52th annual meeting of the Argentine society for clinical investigation. Mar del Plata, Buenos Aires, Argentina; 21-24 November 2007, Medicina (Buenos Aires) 67 (Suppl. III) (2007) 241.
102. A.G. Valdivieso, G.L. Taminelli, M.L. Teiber, M.A. Dankert, T.A. Santa-Coloma, Abstract CB-P82. Down-regulation of the mitochondrial gene ND4 in Cystic Fibrosis. Abstracts of the 42th annual meeting of the Argentine society for biochemistry and molecular biology research. Rosario, Argentina; 12-15 November 2006, Abstract Book 81 (2006).
103. G.L. Taminelli, A.G. Valdivieso, M.L. Teiber, M.A. Dankert, T.A. Santa-Coloma, Abstract CB-P81. ZCD1 is a new mitochondrial-located protein down-regulated in cystic fibrosis. Abstracts of the 42th annual meeting of the Argentine society for biochemistry and molecular biology research. Rosario, Argentina; 12-15 November 2006, Abstracts Book 81 (2006).
104. A.G. Valdivieso, G.L. Taminelli, A.C.F. Tironi Farinati, G.B. Reyes, V. Calabró , M.A. Dankert, T.A. Santa-Coloma, Abstract 451. Regulación del gen ND4 mitocondrial mediada por el canal de cloruro CFTR. Abstracts of the 50th annual meeting of the Argentine society for clinical investigation. Mar del Plata, Buenos Aires, Argentina; 29 November - 2 December 2005, Medicina (Buenos Aires) 65 (Suppl. II) (2005) 167.
105. G.L. Taminelli, A.G. Valdivieso, M.C. Marín, A.C.F. Tironi Farinati, V. Calabró , M.A. Dankert, T.A. Santa-Coloma, Abstract 449. Caracterización de ZCD1, un gen nuclear de localizació n mitocondrial disminuido en Fibrosis Quística. Abstracts of the 50th annual meeting of the Argentine society for clinical investigation. Mar del Plata, Buenos Aires, Argentina; 29 November - 2 December 2005, Medicina (Buenos Aires) 65 (Suppl. II) (2005) 166.
106. A.G. Valdivieso, F. Marcucci, G.L. Taminelli, G.B. Reyes, M.A. Dankert, T.A. Santa-Coloma, Abstract 308. El canal de cloruro CFTR, afectado en Fibrosis Quística, regula la proteína ND4,una subunidad del Complejo I mitocondrial que es clave para el transporte de electrones. Abstracts of the 49th annual meeting of the Argentine society for clinical investigation. Mar del Plata, Buenos Aires, Argentina;16-20 November 2004, Medicina (Buenos Aires) 64 (Suppl. II) (2004) 163.
107. G.L. Taminelli, V. Sotomayor, A.G. Valdivieso, G.B. Reyes, M.A. Dankert, T.A. Santa-Coloma, Abstract 309. KLPX es una proteína de localizació n mitocondrial, con un dominio ''Zn finger'' similar al de algunas kinesinas y cuya expresión estí disminuida en fibrosis quística. Abstracts of the 49th annual meeting of the Argentine society for clinical investigation. Mar delPlata, Buenos Aires, Argentina; 16-20 November2004, Medicina (Buenos Aires) 64 (Suppl. II) (2004) 163.
108. B.L. Shapiro, Evidence for a mitochondrial lesion in cystic fibrosis, Life Sciences 44 (1989) 1327-1334.
109. B.L. Shapiro, Mitochondrial dysfunction, energy expenditure, and cystic fibrosis, Lancet 2 (1988) 289.
110. B.L. Shapiro, L.F. Lam, R.J. Feigal, Mitochondrial NADH dehydrogenase in cystic fibrosis: enzyme kinetics in cultured fibroblasts, American Journal of Human Genetics 34 (1982) 846-852.
111. B.L. Shapiro, R.J. Feigal, L.F. Lam, Mitrochondrial NADH dehydrogenase in cystic fibrosis, Proceedings of the National Academy of Sciences of the United States of America 76 (1979) 2979-2983.
112. R.J. Feigal, B.L. Shapiro, Altered intracellular calcium in fibroblasts from patients with cystic fibrosis and heterozygotes, Pediatric Research 13 (1979) 764-768.
113. R.J. Feigal, B.L. Shapiro, Mitochondrial calcium uptake and oxygen consumption in cystic fibrosis, Nature 278 (1979) 276-277.
114. B.L. Shapiro, L.F. Lam, Intracellular calcium in cystic fibrosis heterozygotes, Life Sciences 40 (1987) 2361-2366.
115. R.J. Feigal, B.L. Shapiro, Cystic fibrosis-a lethal exocrinopathy with altered mitochondrial calcium metabolism, Annals of the New York Academy of Sciences 488 (1986) 82-98.
116. B.L. Shapiro, L.F. Lam, Calcium and age in fibroblasts from control subjects and patients with cystic fibrosis, Science 216 (1982) 417-419.
117. R.J. Feigal, M.S. Tomczyk, B.L. Shapiro, The calcium abnormality in cystic fibrosis mitochondria: relative role of respiration and ATP hydrolysis, Life Sciences 30 (1982) 93-98.
118. B.L. Shapiro, R.J. Feigal, N.J. Laible, M.H. Biros, W.J. Warwick, Doubling time alpha-aminoisobutyrate transport and calcium exchange in cultured fibro-blasts from cystic fibrosis and control subjects, Clinica Chimica Acta 82 (1978) 125-131.
119. B.L. Shapiro, Q.T. Smith, W.J. Warwick, Letter: serum glutathione reductase and cystic fibrosis, Pediatric Research 10 (1976) 250.
120. B.L. Shapiro, Q.T. Smith, W.J. Warick, Serum glutathione reductase and cystic fibrosis, Pediatric Research 9 (1975) 885-888.
121. B.L. Shapiro, Q.T. Smith, A. Martinez, Letter: white-cell glutathione reduc-tase in cystic fibrosis, Lancet 2 (1974) 1020-1021.
122. B.L. Shapiro, Q.T. Smith, W.J. Warwick, Red cell glutathione and glutathione reductase in cystic fibrosis, Proceedings of the Society for Experimental Biology and Medicine 144 (1973) 181-183.
123. I. Antonowicz, W.G. Sippell, H. Shwachman, Cystic fibrosis: lysosomal and mitochondrial enzyme activities of lymphoid cell lines, Pediatric Research 6 (1972) 803-812.
124. P.J. Congdon, J.M. Littlewood, R.K. Aggarwal, H. Shapiro, Glucose 6-phos-phate dehydrogenase deficiency and cystic fibrosis, Postgraduate Medical Journal 57 (1981) 453-454.
125. B.L. Shapiro, L.F. Lam, L.H. Fast, Premature senescence in cultured skin fibroblasts from subjects with cystic fibrosis, Science 203 (1979) 1251-1253.
126. S. Wotman, I.D. Mandel, J. Mercadante, C.R. Denning, Parotid and submax-illary calcium in human cystic fibrosis, Archives of Oral Biology 16 (1971) 663-665.
127. C.C. Lobeck, Cystic fibrosis, in: J.B. Stanbury, J.B. Wyngaarden, D.S. Frederickson (Eds.), The metabolic basis of inherited disease McGraw-Hill, New York, 1972, p. 1605, Chapter 68.
128. S.Y. Botelho, A.M. Goldstein, M.L. Rosenlund, Tear sodium, potassium, chloride, and calcium at various flow rates: children with cystic fibrosis and unaffected siblings with and without corneal staining, Journal of Pediatrics 83 (1973) 601-606.
129. H.M. Allars, J. Blomfield, A.R. Rush, J.M. Brown, Colloid and crystal formation in parotid saliva of cystic fibrosis patients and non-cystic fibrosis subjects. I. Physicochemistry, Pediatric Research 10 (1976) 578-584.
130. J. Blomfield, A.R. Rush, H.M. Allars, J.M. Brown, Parotid gland function in children with cystic fibrosis and child control subjects, Pediatric Research 10 (1976) 574-578.
131. A.A. von Ruecker, R. Bertele, H.K. Harms, Calcium metabolism and cystic fibrosis: mitochondrial abnormalities suggest a modification of the mito-chondrial membrane, Pediatric Research 18 (1984) 594-599.
132. R.L. Waller, W.J. Brattin, D.G. Dearborn, Cytosolic free calcium concentration and intracellular calcium distribution in lymphocytes from cystic fibrosis patients, Life Sciences 35 (1984) 775-781.
133. M.C. Dechecchi, E. Girella, G. Cabrini, G. Berton, The Km of NADH dehy-drogenase is decreased in mitochondria of cystic fibrosis cells, Enzyme 40 (1988) 45-50.
134. J.M. Rommens, S. Zengerling, J. Burns, G. Melmer, B.S. Kerem, N. Plavsic, M. Zsiga, D. Kennedy, D. Markiewicz, R. Rozmahel, J.R. Riordan, M. Buchwald, L.C. Tsui, Identification and regional localization of DNA markers on chromosome 7 for the cloning of the cystic fibrosis gene, American Journal of Human Genetics 43 (1988) 645-663.
135. L. Picci, L. Brentagni, G. Mastella, E. Scarso, P. Pizzochero, P. Mattiazzo, L. Chiandetti, F. Anglani, F. Zacchello, 2D-electrophoresis of mitochondrial proteins from cystic fibrosis patients, Advances in Experimental medicine and Biology 290 (1991) 379-381.
136. K. de Meer, J.A. Jeneson, V.A. Gulmans, J. van der Laag, R. Berger, Efficiency of oxidative work performance of skeletal muscle in patients with cystic fibrosis, Thorax 50 (1995) 980-983.
137. B.J. Day, A.M. van Heeckeren, E. Min, L.W. Velsor, Role for cystic fibrosis transmembrane conductance regulator protein in a glutathione response to bronchopulmonary pseudomonas infection, Infection and Immunity 72 (2004) 2045-2051.
138. P. Liang, A.B. Pardee, Differential display of eukaryotic messenger RNA by means of the polymerase chain reaction, Science 257 (1992) 967-971.
139. E.G. Cafferata, A.M. González-Guerrico, O.H. Pivetta, T.A. Santa-Coloma, Identification by differential display of a mRNA specifically induced by 12-O-tetradecanoylphorbol-13-acetate (TPA) in T84 human colon carcinoma cells, Cell and Molecular Biology (Noisy-le-grand) 42 (1996) 797-804.
140. E.G. Cafferata, A. González-Guerrico, O.H. Pivetta, T.A. Santa-Coloma, Abstract M99. Identificación mediante differential display de genes espe-c óficamente regulados por diferentes factores que afectan la expresión del CFTR(canal de cloruro afectado en Fibrosis Quística). Abstracts of the 31st annual meeting of the Argentine society for biochemistry and molecular biology research. Villa Giardino, Córdoba, Argentina; 13-18 November 1995, Abstracts Book (1995).
141. A.M. González-Guerrico, Expresión de genes asociados a fibrosis quística. Thesis dissertation, School of Exact and Natural Sciences, University of Buenos Aires; 2001, p. 1-120.
142. O. Eidelman, J. Zhang, M. Srivastava, H.B. Pollard, Cystic fibrosis and the use of pharmacogenomics to determine surrogate endpoints for drug discovery, American Journal of Pharmacogenomics 1 (2001) 223-238.
143. H.B. Pollard, O. Eidelman, K.A. Jacobson, M. Srivastava, Pharmacogenomics of cystic fibrosis, Molecular Interventions 1 (2001) 54-63.
144. J.K. Ichikawa, A. Norris, M.G. Bangera, G.K. Geiss, A.B. van't Wout, R.E. Bumgarner, S. Lory, Interaction of Pseudomonas aeruginosa with epithelial cells: identification of differentially regulated genes by expression microarray analysis of human cDNAs, Proceedings of the National Academy of Sciences of the United States of America 97 (2000) 9659-9664.
145. Y. Xu, J.C. Clark, B.J. Aronow, C.R. Dey, C. Liu, J.L. Wooldridge, J.A. Whitsett, Transcriptional adaptation to CFTR deficiency, Journal of Biological Chemistry 12 (2002) 12.
146. Y. Xu, J.C. Clark, B.J. Aronow, C.R. Dey, C. Liu, J.L. Wooldridge, J.A. Whitsett, Transcriptional adaptation to cystic fibrosis transmembrane conductance regulator deficiency, Journal of Biological Chemistry 278 (2003) 7674-7682.
147. V.A. McCarthy, A. Harris, The CFTR gene and regulation of its expression, Pediatric Pulmonology 40 (2005) 1-8.
148. V. Ogilvie, M. Passmore, L. Hyndman, L. Jones, B. Stevenson, A. Wilson, H. Davidson, R.R. Kitchen, R.D. Gray, P. Shah, E.W. Alton, J.C. Davies, D.J. Porteous, A.C. Boyd, Differential global gene expression in cystic fibrosis nasal and bronchial epithelium, Genomics 98 (2011) 327-336.
149. C.E. Jozwik, H.B. Pollard, M. Srivastava, O. Eidelman, Q. Fan, T.N. Darling, P.L. Zeitlin, Antibody microarrays: analysis of cystic fibrosis, Methods in Molecular Biology 823 (2012) 179-200.
150. V. Sotomayor, F. Marcucci, T.A. Santa-Coloma, Abstract 281. CFTR-RG2, un nuevo miembro de la familia de kinesinas regulado por CFTR. Abstracts of the 47th annual meeting of the Argentine society for clinical investigation. Mar del Plata, Buenos Aires, Argentina; 20-23 November 2002, Medicina (Buenos Aires) 62 (2002) 457.
151. V. Sotomayor, F. Marcucci, T.A. Santa-Coloma, Abstract 445. Identificació n de genes regulados por la actividad del CFTR, canal de cloruro afectado en la fibrosis quística (FQ). Abstracts of the 48th annual meeting of the Argentine society for clinical investigation. Mar del Plata, Buenos Aires, Argentina; 19-22 November 2003, Medicina (Buenos Aires) 63 (2003) 631-635.
152. V. Sotomayor, Identificación de genes regulados por la actividad del CFTR, canal de cloruro afectado en la fibrosis quística. Thesis (Lic. in Biological Sciences). Department of Biology, School of Exact and Natural Sciences, University of Buenos Aires; 2003, p. 1-56.
153. F. Marcucci, A.M. González-Guerrico, E. Cafferata, M. Radrizzani, S. Alvarez, T.A. Santa-Coloma, Abstract P031. Identification by differential display of mitochondrial ND4 as a CFTR-dependent gene. Abstracts of the 37th annual meeting of the Argentine society for biochemistry and molecular biology research. Villa Carlos Paz, Có rdoba, Argentina, 2001, Biocell 25 (Supl. II) (2001) 49.
154. F. Marcucci, V. Sotomayor, B. Reyes, T.A. Santa-Coloma, Abstract 282. Identificación mediante display diferencial de ND4 mitocondrial como un gen dependiente de CFTR, canal afectado en fibrosis quística. Abstracts of the 47th annual meeting of the Argentine society for clinical investigation. Mar del Plata, Buenos Aires, Argentina; 20-23 November 2002, Medicina (Buenos Aires) 62 (5) (2002) 457.
155. F. Marcucci, Identificación de genes regulados por CFTR, canal de cloruro afectado en fibrosis quística. Thesis (Lic. in Biology). Department of Biology, School of Exact and Natural Sciences, University of Buenos Aires; 2003, p. 1- 82.
156. A. Marchler-Bauer, A.R. Panchenko, B.A. Shoemaker, P.A. Thiessen, L.Y. Geer, S.H. Bryant, CDD: a database of conserved domain alignments with links to domain three-dimensional structure, Nucleic Acids Research 30 (2002) 281-283.
157. J. Schultz, F. Milpetz, P. Bork, C.P. Ponting, SMART, a simple modular architecture research tool: identification of signaling domains, Proceedings of the National Academy of Sciences of the United States of America 95 (1998) 5857-5864.
158. R.L. Strausberg, E.A. Feingold, L.H. Grouse, J.G. Derge, R.D. Klausner, F.S. Collins, L. Wagner, C.M. Shenmen, G.D. Schuler, S.F. Altschul,
159. E.S. Lander, L.M. Linton, B. Birren, C. Nusbaum, M.C. Zody, J. Baldwin, K. Devon, K. Dewar, M. Doyle, W. FitzHugh, R. Funke, D. Gage, K. Harris, A. Heaford, J. Howland, L. Kann, J. Lehoczky, R. LeVine, P. McEwan, K. McKernan, J. Meldrim, J.P. Mesirov, C. Miranda, W. Morris, J. Naylor,C. Raymond, M. Rosetti, R. Santos, A. Sheridan, C. Sougnez, N. Stange-Thomann, N. Stojanovic, A. Subramanian, D. Wyman, J. Rogers, J. Sulston, R. Ainscough, S. Beck, D. Bentley, J. Burton, C. Clee, N. Carter, A. Coulson, R. Deadman, P. Deloukas, A. Dunham, I. Dunham, R. Durbin, L. French,D. Grafham, S. Gregory, T. Hubbard, S. Humphray, A. Hunt, M. Jones,C. Lloyd, A. McMurray, L. Matthews, S. Mercer, S. Milne, J.C. Mullikin, A. Mungall, R. Plumb, M. Ross, R. Shownkeen, S. Sims, R.H. Waterston, R.K. Wilson, L.W. Hillier, J.D. McPherson, M.A. Marra, E.R. Mardis, L.A. Fulton, A.T. Chinwalla, K.H. Pepin, W.R. Gish, S.L. Chissoe, M.C. Wendl, K.D. Delehaunty, T.L. Miner, A. Delehaunty, J.B. Kramer, L.L. Cook, R.S. Fulton, D.L. Johnson, P.J. Minx, S.W. Clifton, T. Hawkins, E. Branscomb, P. Predki, P. Richardson, S. Wenning, T. Slezak, N. Doggett, J.F. Cheng, A. Olsen, S. Lucas, C. Elkin, E. Uberbacher, M. Frazier, R.A. Gibbs, D.M. Muzny, S.E. Scherer, J.B. Bouck, E.J. Sodergren, K.C. Worley, C.M. Rives, J.H. Gorrell, M.L. Metzker, S.L. Naylor, R.S. Kucherlapati, D.L. Nelson, G.M. Weinstock, Y. Sakaki, A. Fujiyama, M. Hattori, T. Yada, A. Toyoda, T. Itoh, C. Kawagoe, H. Watanabe, Y. Totoki, T. Taylor, J. Weissenbach, R. Heilig, W. Saurin, F. Artiguenave, P. Brottier, T. Bruls,E. Pelletier, C. Robert, P. Wincker, D.R. Smith, L. Doucette-Stamm, M. Rubenfield, K. Weinstock, H.M. Lee, J. Dubois, A. Rosenthal, M. Platzer, G. Nyakatura, S. Taudien, A. Rump, H. Yang, J. Yu, J. Wang, G. Huang, J. Gu, L. Hood, L. Rowen, A. Madan, S. Qin, R.W. Davis, N.A. Federspiel, A.P. Abola, M.J. Proctor, R.M. Myers, J. Schmutz, M. Dickson, J. Grimwood, D.R. Cox, M.V. Olson, R. Kaul, N. Shimizu, K. Kawasaki, S. Minoshima, G.A. Evans, M. Athanasiou, R. Schultz, B.A. Roe, F. Chen, H. Pan, J. Ramser, H. Lehrach, R. Reinhardt, W.R. McCombie, M. de la Bastide, N. Dedhia, H. Blocker, K. Hornischer, G. Nordsiek, R. Agarwala, L. Aravind, J.A. Bailey, A. Bateman, S. Batzoglou, E. Birney, P. Bork, D.G. Brown, C.B. Burge, L. Cerutti, H.C. Chen,D. Church, M. Clamp, R.R. Copley, T. Doerks, S.R. Eddy, E.E. Eichler, T.S. Furey,J. Galagan, J.G. Gilbert, C. Harmon, Y. Hayashizaki, D. Haussler, H. Hermjakob, K. Hokamp, W. Jang, L.S. Johnson, T.A. Jones, S. Kasif, A. Kaspryzk, S. Kennedy, W.J. Kent, P. Kitts, E.V. Koonin, I. Korf, D. Kulp, D. Lancet, T.M. Lowe, A. McLysaght, T. Mikkelsen, J.V. Moran, N. Mulder, V.J. Pollara, C.P. Ponting, G. Schuler, J. Schultz, G. Slater, A.F. Smit, E. Stupka, J. Szustakowski, D. Thierry-Mieg, J. Thierry-Mieg, L. Wagner, J. Wallis, R. Wheeler, A. Williams, Y.I. Wolf, K.H. Wolfe, S.P. Yang, R.F. Yeh,
160. G. Derosa, P. Maffioli, Peroxisome proliferator-activated receptor-gamma (PPAR-gamma) agonists on glycemic control, lipid profile and cardiovascular risk, Current Molecular Pharmacology 5 (2) (2011) 272-281.
161. J.R. Colca, W.G. McDonald, D.J. Waldon, J.W. Leone, J.M. Lull, C.A. Bannow, E.T. Lund, W.R. Mathews, Identification of a novel mitochondrial protein (''mitoNEET'') cross-linked specifically by a thiazolidinedione photoprobe, American Journal of Physiology-Endocrinology and Metabolism 286 (2004) E252-260.
162. X. Hou, R. Liu, S. Ross, E.J. Smart, H. Zhu, W. Gong, Crystallographic studies of human MitoNEET, Journal of Biological Chemistry 282 (46) (2007) 33242-33246.
163. J. Lin, T. Zhou, K. Ye, J. Wang, Crystal structure of human mitoNEET reveals distinct groups of iron sulfur proteins, Proceedings of the National Academy of Sciences of the United States of America 104 (2007) 14640-14645.
164. M.L. Paddock, S.E. Wiley, H.L. Axelrod, A.E. Cohen, M. Roy, E.C. Abresch, D. Capraro, A.N. Murphy, R. Nechushtai, J.E. Dixon, P.A. Jennings, MitoNEET is a uniquely folded 2Fe-2S outer mitochondrial membrane protein stabilized by pioglitazone, Proceedings of the National Academy of Sciences of the United States of America 104 (2007) 14342-14347.
165. S.E. Wiley, M.L. Paddock, E.C. Abresch, L. Gross, P. van der Geer, R. Nechushtai, A.N. Murphy, P.A. Jennings, J.E. Dixon, The outer mitochon-drial membrane protein mitoNEET contains a novel redox-active 2Fe-2S cluster, Journal of Biological Chemistry 282 (2007) 23745-23749.
166. S.E. Wiley, A.N. Murphy, S.A. Ross, P. van der Geer, J.E. Dixon, MitoNEET is an iron-containing outer mitochondrial membrane protein that regulates oxidative capacity, Proceedings of the National Academy of Sciences of the United States of America 104 (2007) 5318-5323.
167. G. Tan, A.P. Landry, R. Dai, L. Wang, J. Lu, H. Ding, Competition of zinc ion for the [2Fe-2S] cluster binding site in the diabetes drug target protein mitoNEET, Biometals 25 (6) (2012) 1177-1184.
168. R.M. Bieganski, M.L. Yarmush, Novel ligands that target the mitochondrial membrane protein mitoNEET, Journal of Molecular Graphics and Modelling 29 (2011) 965-973.
169. J.A. Zuris, Y. Harir, A.R. Conlan, M. Shvartsman, D. Michaeli, S. Tamir, M.L. Paddock, J.N. Onuchic, R. Mittler, Z.I. Cabantchik, P.A. Jennings, R. Nechushtai, Facile transfer of [2Fe-2S] clusters from the diabetes drug target mitoNEET to an apo-acceptor protein, Proceedings of the National Academy of Sciences of the United States of America 108 (32) (2011) 13047-13052.
170. T. Zhou, J. Lin, Y. Feng, J. Wang, Binding of reduced nicotinamide adenine dinucleotide phosphate destabilizes the iron-sulfur clusters of human mitoNEET, Biochemistry 49 (2010) 9604-9612.
171. J.A. Zuris, S.S. Ali, H. Yeh, T.A. Nguyen, R. Nechushtai, M.L. Paddock, P.A. Jennings, NADPH inhibits [2Fe-2S] cluster protein transfer from diabetes drug target MitoNEET to an apo-acceptor protein, Journal of Biological Chemistry 287 (2012) 11649-11655.
172. A. Chomyn, Mitochondrial genetic control of assembly and function of complex I in mammalian cells, Journal of Bioenergetics and Biomembranes 33 (2001) 251-257.
173. J. Carroll, I.M. Fearnley, J.M. Skehel, R.J. Shannon, J. Hirst, J.E. Walker, Bovine complex I is a complex of 45 different subunits, Journal of Biological Chemistry 281 (2006) 32724-32727.
174. G. Hofhaus, G. Attardi, Lack of assembly of mitochondrial DNA-encoded subunits of respiratory NADH dehydrogenase and loss of enzyme activity in a human cell mutant lacking the mitochondrial ND4 gene product, EMBO Journal 12 (1993) 3043-3048.
175. G. Hofhaus, G. Attardi, Efficient selection and characterization of mutants of a human cell line which are defective in mitochondrial DNA-encoded subunits of respiratory NADH dehydrogenase, Molecular and Cellular Biology 15 (1995) 964-974.
176. Y. Bai, P. Hajek, A. Chomyn, E. Chan, B.B. Seo, A. Matsuno-Yagi, T. Yagi, G. Attardi, Lack of complex I activity in human cells carrying a mutation in MtDNA-encoded ND4 subunit is corrected by the Saccharomyces cerevisiae NADH-quinone oxidoreductase (NDI1) gene, Journal of Biological Chemistry 276 (2001) 38808-38813.
177. P. Cardol, R.F. Matagne, C. Remacle, Impact of mutations affecting ND mitochondria-encoded subunits on the activity and assembly of complex I in Chlamydomonas. Implication for the structural organization of the enzyme, Journal of Molecular Biology 319 (2002) 1211-1221.
178. I. Bourges, C. Ramus, B. Mousson de Camaret, R. Beugnot, C. Remacle, P. Cardol, G. Hofhaus, J.P. Issartel, Structural organization of mitochondrial human complex I: role of the ND4 and ND5 mitochondria-encoded subunits and interaction with prohibitin, Biochemical Journal 383 (2004) 491-499.
179. X. Qi, L. Sun, A.S. Lewin, W.W. Hauswirth, J. Guy, The mutant human ND4 subunit of complex I induces optic neuropathy in the mouse, Investigative Ophthalmology and Visual Science 48 (2007) 1-10.
180. A. Baracca, G. Solaini, G. Sgarbi, G. Lenaz, A. Baruzzi, A.H. Schapira, A. Martinuzzi, V. Carelli, Severe impairment of complex I-driven adenosine triphosphate synthesis in leber hereditary optic neuropathy cybrids, Archives of Neurology 62 (2005) 730-736.
181. A. Majander, K. Huoponen, M.L. Savontaus, E. Nikoskelainen, M. Wikstrom, Electron transfer properties of NADH:ubiquinone reductase in the ND1/ 3460 and the ND4/11778 mutations of the Leber hereditary optic neuror-etinopathy (LHON), FEBS Letters 292 (1991) 289-292.
182. M. Kelly-Aubert, S. Trudel, J. Fritsch, T. Nguyen-Khoa, M. Baudouin-Legros, S. Moriceau, L. Jeanson, F. Djouadi, C. Matar, M. Conti, M. Ollero, F. Brouillard, A. Edelman, GSH monoethyl ester rescues mitochondrial defects in cystic fibrosis models, Human Molecular Genetics 20 (2011) 2745-2759.
183. F. Antigny, C. Norez, F. Becq, C. Vandebrouck, CFTR and Ca signaling in cystic fibrosis, Frontiers in Pharmacology 2 (2011) 67.
184. G.N. Donnell, R.S. Cleland, Intestinal atresia or stenosis in the newborn associated with fibrocystic disease of the pancreas, California Medicine 94 (1961) 165-170.
185. J. Marmar, G.J. Barbero, M.S. Sibinga, The pattern of parotid gland secretion in cystic fibrosis of the pancreas, Gastroenterology 50 (1966) 551-556.
186. J. Blomfield, J. Dascalu, E.W. van Lennep, J.M. Brown, Hypersecretion of zymogen granules in the pathogenesis of cystic fibrosis, Gut 14 (1973) 558-565.
187. F. Antigny, N. Girardin, D. Raveau, M. Frieden, F. Becq, C. Vandebrouck, Dysfunction of mitochondria Ca2p uptake in cystic fibrosis airway epithelial cells, Mitochondrion 9 (2009) 232-241.
188. C.M. Ribeiro, A.M. Paradiso, M.A. Carew, S.B. Shears, R.C. Boucher, Cystic fibrosis airway epithelial Ca2p i signaling: the mechanism for the larger agonist-mediated Ca2p i signals in human cystic fibrosis airway epithelia, Journal of Biological Chemistry 280 (2005) 10202-10209.
189. O. Tabary, E. Boncoeur, R. de Martin, R. Pepperkok, A. Clement, C. Schultz, J. Jacquot, Calcium-dependent regulation of NF-(kappa)B activation in cystic fibrosis airway epithelial cells, Cellular Signalling 18 (2006) 652-660.
190. F. Galli, A. Battistoni, R. Gambari, A. Pompella, A. Bragonzi, F. Pilolli, L. Iuliano, M. Piroddi, M.C. Dechecchi, G. Cabrini, Oxidative stress and antioxidant therapy in cystic fibrosis, Biochimica et Biophysica Acta 1822 (2012) 690-713.
191. S. l'Hoste, A. Chargui, R. Belfodil, E. Corcelle, C. Duranton, I. Rubera, C. Poujeol, B. Mograbi, M. Tauc, P. Poujeol, CFTR mediates apoptotic volume decrease and cell death by controlling glutathione efflux and ROS production in cultured mice proximal tubules, American Journal of Physiology-Renal Physiology 298 (2010) F435-453.
192. M. Rottner, S. Tual-Chalot, H.A. Mostefai, R. Andriantsitohaina, J.M. Freyssinet, M.C. Martinez, Increased oxidative stress induces apoptosis in human cystic fibrosis cells, PLoS One 6 (2011) e24880.
193. L.W. Velsor, C. Kariya, R. Kachadourian, B.J. Day, Mitochondrial oxidative stress in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice, American Journal of Respiratory Cell and Molecular Biology 35 (2006) 579-586.
194. Y. Zhang, X. Li, H. Grassme, G. Doring, E. Gulbins, Alterations in ceramide concentration and pH determine the release of reactive oxygen species by Cftr-deficient macrophages on infection, Journal of Immunology 184 (2010) 5104-5111.
195. A. Luciani, V.R. Villella, S. Esposito, N. Brunetti-Pierri, D. Medina, C. Settembre, M. Gavina, L. Pulze, I. Giardino, M. Pettoello-Mantovani, M. D'Apolito, S. Guido, E. Masliah, B. Spencer, S. Quaratino, V. Raia, A. Ballabio, L. Maiuri, Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition, Nature Cell Biology 12 (2010) 863-875.
196. F. Thevenod, Multifaceted CFTR: novel role in ROS signaling and apoptotic cell death-a commentary on ''CFTR mediates cadmium-induced apoptosis through modulation of ROS levels in mouse proximal tubule cells'', Free Radical Biology and Medicine 46 (2009) 1014-1016.
197. S. L'Hoste, A. Chargui, R. Belfodil, C. Duranton, I. Rubera, B. Mograbi, C. Poujeol, M. Tauc, P. Poujeol, CFTR mediates cadmium-induced apoptosis through modulation of ROS level in mouse proximal tubule cells, Free Radical Biology and Medicine 46 (2009) 1017-1031.
198. S. Ahmad, D.P. Nichols, M. Strand, R.C. Rancourt, S.H. Randell, C.W. White, A. Ahmad, SERCA2 regulates non-CF and CF airway epithelial cell response to ozone, PLoS One 6 (2011) e27451.
199. O. Kamdar, W. Le, J. Zhang, A.J. Ghio, G.D. Rosen, D. Upadhyay, Air pollution induces enhanced mitochondrial oxidative stress in cystic fibrosis airway epithelium, FEBS Letters 582 (2008) 3601-3606.
200. V. Jendrossek, H. Grassme, I. Mueller, F. Lang, E. Gulbins, Pseudomonas aeruginosa-induced apoptosis involves mitochondria and stress-activated protein kinases, Infection and Immunity 69 (2001) 2675-2683.
201. M.W. Cleeter, J.M. Cooper, A.H. Schapira, Irreversible inhibition of mito-chondrial complex I by 1-methyl-4-phenylpyridinium: evidence for free radical involvement, Journal of Neurochemistry 58 (1992) 786-789.
202. L.A. Esposito, S. Melov, A. Panov, B.A. Cottrell, D.C. Wallace, Mitochondrial disease in mouse results in increased oxidative stress, Proceedings of the National Academy of Sciences of the United States of America 96 (1999) 4820-4825.
203. F.Q. Liang, B.F. Godley, Oxidative stress-induced mitochondrial DNA damage in human retinal pigment epithelial cells: a possible mechanism for RPE aging and age-related macular degeneration, Experimental Eye Research 76 (2003) 397-403.
204. B. Van Houten, V. Woshner, J.H. Santos, Role of mitochondrial DNA in toxic responses to oxidative stress, DNA Repair (Amst) 5 (2006) 145-152.
205. G. Escames, L.C. Lopez, J.A. Garcia, L. Garcia-Corzo, F. Ortiz, D. Acuna-Castroviejo, Mitochondrial DNA and inflammatory diseases, Human Genetics 131 (2012) 161-173.
206. A.S. Patel, D. Morse, A.M. Choi, Regulation and functional significance of autophagy in respiratory cell biology and disease, American Journal of Respiratory Cell and Molecular Biology 48 (1) (2012) 1-9.
207. K.A. Markossian, B.I. Kurganov, Protein folding, misfolding, and aggregation. Formation of inclusion bodies and aggresomes, Biochemistry (Mosc) 69 (2004) 971-984.
208. A. Luciani, V.R. Villella, S. Esposito, N. Brunetti-Pierri, D.L. Medina, C. Settembre, M. Gavina, V. Raia, A. Ballabio, L. Maiuri, Cystic fibrosis: a disorder with defective autophagy, Autophagy 7 (2011) 104-106.
209. B.A. Abdulrahman, A.A. Khweek, A. Akhter, K. Caution, S. Kotrange, D.H. Abdelaziz, C. Newland, R. Rosales-Reyes, B. Kopp, K. McCoy, R. Montione, L.S. Schlesinger, M.A. Gavrilin, M.D. Wewers, M.A. Valvano, A.O. Amer, Autophagy stimulation by rapamycin suppresses lung inflammation and infection by Burkholderia cenocepacia in a model of cystic fibrosis, Autophagy 7 (2011) 1359-1370.
210. A. Luciani, V.R. Villella, S. Esposito, M. Gavina, I. Russo, M. Silano, S. Guido, M. Pettoello-Mantovani, R. Carnuccio, B. Scholte, A. De Matteis, M.C. Maiuri, V. Raia, A. Luini, G. Kroemer, L. Maiuri, Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on DeltaF508 cystic fibrosis transmembrane conductance regulator, Autophagy 8 (2012) 1657-1672.
211. L. Gao, K.J. Kim, J.R. Yankaskas, H.J. Forman, Abnormal glutathione transport in cystic fibrosis airway epithelia, American Journal of Physiology 277 (1999) L113-118.
212. P. Linsdell, J.W. Hanrahan, Glutathione permeability of CFTR, American Journal of Physiology 275 (1998) C323-326.
213. L.W. Velsor, A. van Heeckeren, B.J. Day, Antioxidant imbalance in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice, American Journal of Physiology Lung Cellular and Molecular Physiology 281 (2001) L31-38.
214. J.C. Fernandez-Checa, C. Garcia-Ruiz, M. Ookhtens, N. Kaplowitz, Impaired uptake of glutathione by hepatic mitochondria from chronic ethanol-fed rats. Tracer kinetic studies in vitro and in vivo and susceptibility to oxidant stress, Journal of Clinical Investigation 87 (1991) 397-405.
215. J. Martensson, A. Jain, W. Frayer, A. Meister, Glutathione metabolism in the lung: inhibition of its synthesis leads to lamellar body and mitochondrial defects, Proceedings of the National Academy of Sciences of the United States of America 86 (1989) 5296-5300.
216. J. Martensson, A. Meister, Mitochondrial damage in muscle occurs after marked depletion of glutathione and is prevented by giving glutathione monoester, Proceedings of the National Academy of Sciences of the United States of America 86 (1989) 471-475.
217. S. Trudel, M. Kelly, J. Fritsch, T. Nguyen-Khoa, P. Therond, M. Couturier, M. Dadlez, J. Debski, L. Touqui, B. Vallee, M. Ollero, A. Edelman, F. Brouillard, Peroxiredoxin 6 fails to limit phospholipid peroxidation in lung from Cftr-knockout mice subjected to oxidative challenge, PLoS One 4 (2009) e6075.
218. Y. Wang, Y. Manevich, S.I. Feinstein, A.B. Fisher, Adenovirus-mediated transfer of the 1-cys peroxiredoxin gene to mouse lung protects against hyperoxic injury, American Journal of Physiology Lung Cellular and Molecular Physiology 286 (2004) L1188-1193.
219. Y. Wang, S.A. Phelan, Y. Manevich, S.I. Feinstein, A.B. Fisher, Transgenic mice overexpressing peroxiredoxin 6 show increased resistance to lung injury in hyperoxia, American Journal of Respiratory Cell and Molecular Biology 34 (2006) 481-486.
220. A.B. Fisher, C. Dodia, Y. Manevich, J.W. Chen, S.I. Feinstein, Phospholipid hydroperoxides are substrates for non-selenium glutathione peroxidase, Journal of Biological Chemistry 274 (1999) 21326-21334.
221. A. Madarasi, A. Lugassi, E. Greiner, K. Holics, L. Biro, E. Mozsary, Antioxidant status in patients with cystic fibrosis, Annals of Nutrition and Metabolism 44 (2000) 207-211.
222. S.S. Percival, E. Bowser, M. Wagner, Reduced copper enzyme activities in blood cells of children with cystic fibrosis, American Journal of Clinical Nutrition 62 (1995) 633-638.
223. S.S. Percival, G.P. Kauwell, E. Bowser, M. Wagner, Altered copper status in adult men with cystic fibrosis, Journal of the American College of Nutrition 18 (1999) 614-619.
224. A.M. Gallagher, R.A. Gottlieb, Proliferation, not apoptosis, alters epithelial cell migration in small intestine of CFTR null mice, American Journal of Physiology-Gastrointestinal and Liver Physiology 281 (2001) G681-687.
225. V. Jendrossek, S. Fillon, C. Belka, I. Muller, B. Puttkammer, F. Lang, Apoptotic response of Chang cells to infection with Pseudomonas aeruginosa strains PAK and PAO-I: molecular ordering of the apoptosis signaling cascade and role of type IV pili, Infection and Immunity 71 (2003) 2665-2673.
226. S. Kirschnek, E. Gulbins, Phospholipase A2 functions in Pseudomonas aeruginosa-induced apoptosis, Infection and Immunity 74 (2006) 850-860.
227. M. Rottner, C. Kunzelmann, M. Mergey, J.M. Freyssinet, M.C. Martinez, Exaggerated apoptosis and NF-kappaB activation in pancreatic and tracheal cystic fibrosis cells, FASEB Journal 21 (2007) 2939-2948.
228. C. Schwarzer, Z. Fu, M. Patanwala, L. Hum, M. Lopez-Guzman, B. Illek, W. Kong, S.V. Lynch, T.E. Machen, Pseudomonas aeruginosa biofilm-asso-ciated homoserine lactone C12 rapidly activates apoptosis in airway epithelia, Cellular Microbiology 14 (2012) 698-709.
229. H. Barriere, C. Poujeol, M. Tauc, J.M. Blasi, L. Counillon, P. Poujeol, CFTR modulates programmed cell death by decreasing intracellular pH in Chinese hamster lung fibroblasts, American Journal of Physiology Cell Physiology 281 (2001) C810-824.
230. R. Bals, D.J. Weiner, J.M. Wilson, The innate immune system in cystic fibrosis lung disease, Journal of Clinical Investigation 103 (1999) 303-307.
231. M. Conese, Cystic fibrosis and the innate immune system: therapeutic implications, Endocrine, Metabolic and Immune Disorders Drug Targets 11 (2011) 8-22.
232. G. Kronborg, M.B. Hansen, M. Svenson, A. Fomsgaard, N. Hoiby, K. Bendtzen, Cytokines in sputum and serum from patients with cystic fibrosis and chronic Pseudomonas aeruginosa infection as markers of destructive inflammation in the lungs, Pediatric Pulmonology 15 (1993) 292-297.
233. G. Kronborg, Lipopolysaccharide (LPS), LPS-immune complexes and cyto-kines as inducers of pulmonary inflammation in patients with cystic fibrosis and chronic Pseudomonas aeruginosa lung infection, APMIS Supplementum 50 (1995) 1-30.
234. Y. Dai, T.P. Dean, M.K. Church, J.O. Warner, J.K. Shute, Desensitisation of neutrophil responses by systemic interleukin 8 in cystic fibrosis, Thorax 49 (1994) 867-871.
235. A. Schuster, A. Haarmann, V. Wahn, Cytokines in neutrophil-dominated airway inflammation in patients with cystic fibrosis, European Archives of Oto-Rhino-Laryngology Supplement 1 (1995) S59-60.
236. P.S. Salva, N.A. Doyle, L. Graham, H. Eigen, C.M. Doerschuk, TNF-alpha, IL-8, soluble ICAM-1, and neutrophils in sputum of cystic fibrosis patients, Pediatric Pulmonology 21 (1996) 11-19.
237. D.Y. Koller, I. Nething, J. Otto, R. Urbanek, I. Eichler, Cytokine concentrations in sputum from patients with cystic fibrosis and their relation to eosinophil activity, American Journal of Respiratory and Critical Care Medicine 155 (1997) 1050-1054.
238. R.W. Palfreyman, M.L. Watson, C. Eden, A.W. Smith, Induction of biologically active interleukin-8 from lung epithelial cells by Burkholderia (Pseudomo-nas) cepacia products, Infection and Immunity 65 (1997) 617-622.
239. L.S. Nixon, B. Yung, S.C. Bell, J.S. Elborn, D.J. Shale, Circulating immunor-eactive interleukin-6 in cystic fibrosis, American Journal of Respiratory and Critical Care Medicine 157 (1998) 1764-1769.
240. F. Karpati, F.L. Hjelte, B. Wretlind, TNF-alpha and IL-8 in consecutive sputum samples from cystic fibrosis patients during antibiotic treatment, Scandinavian Journal of Infectious Diseases 32 (2000) 75-79.
241. L.P. McGarvey, K. Dunbar, S.L. Martin, V. Brown, J. Macmahon, M. Ennis, J.S. Elborn, Cytokine concentrations and neutrophil elastase activity in bronchoalveolar lavage and induced sputum from patients with cystic fibrosis, mild asthma and healthy volunteers, Journal of Cystic Fibrosis 1 (2002) 269-275.
242. C. Colombo, D. Costantini, A. Rocchi, L. Cariani, M.L. Garlaschi, S. Tirelli, G. Calori, E. Copreni, M. Conese, Cytokine levels in sputum of cystic fibrosis patients before and after antibiotic therapy, Pediatric Pulmonology 40 (2005) 15-21.
243. S.D. Sagel, R.K. Kapsner, I. Osberg, Induced sputum matrix metalloprotei-nase-9 correlates with lung function and airway inflammation in children with cystic fibrosis, Pediatric Pulmonology 39 (2005) 224-232.
244. K.J. Mackerness, G.R. Jenkins, A. Bush, P.J. Jose, Characterisation of the range of neutrophil stimulating mediators in cystic fibrosis sputum, Thorax 63 (2008) 614-620.
245. D. Nichols, J. Chmiel, M. Berger, Chronic inflammation in the cystic fibrosis lung: alterations in inter- and intracellular signaling, Clinical Reviews in Allergy and Immunology 34 (2008) 146-162.
246. S.L. Martin, K.L. Moffitt, A. McDowell, C. Greenan, R.J. Bright-Thomas, A.M. Jones, A.K. Webb, J.S. Elborn, Association of airway cathepsin B and S with inflammation in cystic fibrosis, Pediatric Pulmonology 45 (2010) 860-868.
247. T.S. Cohen, A. Prince, Cystic fibrosis: a mucosal immunodeficiency syndrome, Nature Medicine 18 (2012) 509-519.
248. K.A. Becker, B. Henry, R. Ziobro, B. Tummler, E. Gulbins, H. Grassme, Role of CD95 in pulmonary inflammation and infection in cystic fibrosis, Journal of Molecular Medicine (Berlin) 90 (9) (2012) 1011-1023.
249. X. Su, M.R. Looney, H.E. Su, J.W. Lee, Y. Song, M.A. Matthay, Role of CFTR expressed by neutrophils in modulating acute lung inflammation and injury in mice, Inflammation Research 60 (2011) 619-632.
250. N. Regamey, P.K. Jeffery, E.W. Alton, A. Bush, J.C. Davies, Airway remodelling and its relationship to inflammation in cystic fibrosis, Thorax 66 (2011) 624-629.
251. T.P. Carroll, C.M. Greene, C.C. Taggart, N.G. McElvaney, S.J. ONeill, Inter-leukin-1, neutrophil elastase, and lipopolysaccharide: key pro-inflammatory stimuli regulating inflammation in cystic fibrosis, Current Respiratory Medicine Reviews 1 (2005) 43-67.
252. A. Meissner, J. Yang, J.T. Kroetsch, M. Sauve, H. Dax, A. Momen, M.H. Noyan-Ashraf, S. Heximer, M. Husain, D. Lidington, S.S. Bolz, Tumor necrosis factor-a-mediated downregulation of the cystic fibrosis transmembrane conductance regulator drives pathological sphingosine-1-phosphate signaling in a mouse model of heart failure., Circulation 125 (22) (2012) 2739-2750.
253. H. Nakamura, K. Yoshimura, G. Bajocchi, B.C. Trapnell, A. Pavirani, R.G. Crystal, Tumor necrosis factor modulation of expression of the cystic fibrosis transmembrane conductance regulator gene, FEBS Letters 314 (1992) 366-370.
254. J.S. Elborn, D. Norman, F.M. Delamere, D.J. Shale, In vitro tumor necrosis factor-alpha secretion by monocytes from patients with cystic fibrosis, American Journal of Respiratory Cell and Molecular Biology 6 (1992) 207-211.
255. M.A. Brown, W.J. Morgan, P.R. Finley, P. Scuderi, Circulating levels of tumor necrosis factor and interleukin-1 in cystic fibrosis, Pediatric Pulmonology 10 (1991) 86-91.
256. N. Vij, S. Mazur, P.L. Zeitlin, CFTR is a negative regulator of NFkappaB mediated innate immune response, PLoS One 4 (2009) e4664.
257. M.J. Lopez-Armada, B. Carames, M.A. Martin, B. Cillero-Pastor, M. Lires-Dean, I. Fuentes-Boquete, J. Arenas, F.J. Blanco, Mitochondrial activity is modulated by TNFalpha and IL-1beta in normal human chondrocyte cells, Osteoarthritis and Cartilage 14 (2006) 1011-1022.
258. M. Clauzure, A.G. Valdivieso, F. Sánchez, G.L. Taminelli, E.S. Pagano, M.M. Massip Copiz, G. Schulman, M.L. Teiber, T.A. Santa-Coloma, Abstract 182. Efecto de IL-1b en la regulación de la actividad mitocondrial en Fibrosis Quística. Abstracts of the 55th annual meeting of the Argentine society for clinical research. Mar del Plata, Buenos Aires, Argentina; 17-20 November 2010, Medicina (Buenos Aires) 70 (Suppl. II) (2010) 107.
259. T.R. Bartling, M.L. Drumm, Oxidative stress causes IL8 promoter hyperace-tylation in cystic fibrosis airway cell models, American Journal of Respiratory Cell and Molecular Biology 40 (2009) 58-65.
260. P. Montemurro, M.A. Mariggio, G. Barbuti, A. Cassano, A. Vincenti, G. Serio, L. Guerra, A. Diana, T. Santostasi, A. Polizzi, R. Fumarulo, V. Casavola, A. Manca, M. Conese, Increase in interleukin-8 production from circulating neutrophils upon antibiotic therapy in cystic fibrosis patients, Journal of Cystic Fibrosis 11 (6) (2012) 518-524.
261. A. Saadane, J. Eastman, M. Berger, T.L. Bonfield, Parthenolide inhibits ERK and AP-1 which are dysregulated and contribute to excessive IL-8 expression and secretion in cystic fibrosis cells, Journal of Inflammation (London) 8 (2011) 26.
262. S.K.Kaza, S.McClean, M.Callaghan, IL-8 released from human lung epithelial cells induced by cystic fibrosis pathogens Burkholderia cepacia complex affects the growth and intracellular survival of bacteria, International Journal of Medical Microbiology 301(2011) 26-33.
263. L. Brandolini, R. Sergi, G. Caselli, D. Boraschi, M. Locati, S. Sozzani, R. Bertini, Interleukin-1 beta primes interleukin-8-stimulated chemotaxis and elastase release in human neutrophils via its type I receptor, European Cytokine Network 8 (1997) 173-178.
264. T.L. Bonfield, J.R. Panuska, M.W. Konstan, K.A. Hilliard, J.B. Hilliard, H. Ghnaim , M. Berger, Inflammatory cytokines in cystic fibrosis lungs, American Journal of Respiratory and Critical Care Medicine 152 (1995) 2111-2118.
265. T.P. Dean, Y. Dai, J.K. Shute, M.K. Church, J.O. Warner, Interleukin-8 concentrations are elevated in bronchoalveolar lavage, sputum, and sera of children with cystic fibrosis, Pediatric Research 34 (1993) 159-161.
266. S. Mizunoe, T. Shuto, S. Suzuki, C. Matsumoto, K. Watanabe, K. Ueno-Shuto, M.A. Suico, K. Onuki, D.C. Gruenert, H. Kai, Synergism Between Interleukin (IL)-17 and Toll-like Receptor 2 and 4 Signals to Induce IL-8 Expression in Cystic Fibrosis Airway Epithelial Cells, Journal of Pharmacological Sciences 118 (2012) 512-520.
267. L. Derycke, N. Zhang, G. Holtappels, T. Dutre, C. Bachert, IL-17A as a regulator of neutrophil survival in nasal polyp disease of patients with and without cystic fibrosis, Journal of Cystic Fibrosis 11 (2012) 193-200.
268. H.L. Tan, N. Regamey, S. Brown, A. Bush, C.M. Lloyd, J.C. Davies, The Th17 pathway in cystic fibrosis lung disease, American Journal of Respiratory and Critical Care Medicine 184 (2011) 252-258.
269. M. Brodlie, M.C. McKean, G.E. Johnson, A.E. Anderson, C.M. Hilkens, A.J. Fisher, P.A. Corris, J.L. Lordan, C. Ward, Raised interleukin-17 is immunolocalised to neutrophils in cystic fibrosis lung disease, European Respiratory Journal 37 (2011) 1378-1385.
270. Y. Chen, P. Thai, Y.H. Zhao, Y.S. Ho, M.M. DeSouza, R. Wu, Stimulation of airway mucin gene expression by interleukin (IL)-17 through IL-6 paracrine/autocrine loop, Journal of Biological Chemistry 278 (2003) 17036-17043.
271. P. Brennan, L.A. O'Neill, Effects of oxidants and antioxidants on nuclear factor kappa B activation in three different cell lines: evidence against a universal hypothesis involving oxygen radicals, Biochimica et Biophysica Acta 1260 (1995) 167-175.
272. S. Brennan, Innate immune activation and cystic fibrosis, Paediatric Respiratory Reviews 9 (2008) 271-279. (quiz 279-280).
273. M. Filosto, P. Tonin, G. Vattemi, C. Savio, N. Rizzuto, G. Tomelleri, Transcription factors c-Jun/activator protein-1 and nuclear factor-kappa B in oxida-tive stress response in mitochondrial diseases, Neuropathology and Applied Neurobiology 29 (2003) 52-59.
274. M.W. Konstan, M. Berger, Current understanding of the inflammatory process in cystic fibrosis: onset and etiology, Pediatric Pulmonology 24 (1997) 137-142. (discussion 159-161).
275. J. Yang, J.P. Eiserich, C.E. Cross, B.M. Morrissey, B.D. Hammock, Metabolomic profiling of regulatory lipid mediators in sputum from adult cystic fibrosis patients, Free Radical Biology and Medicine 53 (1) (2012) 160-171.
276. P.B. van de Weert-van Leeuwen, M.G. Slieker, H.J. Hulzebos, C.L. Kruitwagen, C.K. van der Ent, H.G. Arets, Chronic infection and inflammation affect exercise capacity in cystic fibrosis, European Respiratory Journal 39 (2012) 893-898.
277. N. Regamey, L. Tsartsali, T.N. Hilliard, O. Fuchs, H.L. Tan, J. Zhu, Y.S. Qiu, E.W. Alton, P.K. Jeffery, A. Bush, J.C. Davies, Distinct patterns of inflammation in the airway lumen and bronchial mucosa of children with cystic fibrosis, Thorax 67 (2012) 164-170.
278. C. Rebeyrol, V. Saint-Criq, L. Guillot, L. Riffault, H. Corvol, K. Chadelat, D.W. Ray, A. Clement, O. Tabary, P. Le Rouzic, Glucocorticoids reduce inflammation in cystic fibrosis bronchial epithelial cells, Cellular signalling 24 (2012) 1093-1099.
279. H. Everett, G. McFadden, Apoptosis: an innate immune response to virus infection, Trends in Microbiology 7 (1999) 160-165.
280. S.M. McWhirter, B.R. Tenoever, T. Maniatis, Connecting mitochondria and innate immunity, Cell 122 (2005) 645-647.
281. R.B. Seth, L. Sun, Z.J. Chen, Antiviral innate immunity pathways, Cell Research 16 (2006) 141-147.
282. I. Scott, Mitochondrial factors in the regulation of innate immunity, Microbes and Infection 11 (2009) 729-736.
283. V. Temkin, M. Karin, From death receptor to reactive oxygen species and c-Jun N-terminal protein kinase: the receptor-interacting protein 1 odyssey, Immunological Reviews 220 (2007) 8-21.
284. A. Rudiger, M. Stotz, M. Singer, Cellular processes in sepsis, Swiss Medical Weekly 138 (2008) 629-634.
285. G. Yeretssian, K. Labbe, M. Saleh, Molecular regulation of inflammation and cell death, Cytokine 43 (2008) 380-390.
286. J.P. Ting, J.A. Duncan, Y. Lei, How the noninflammasome NLRs function in the innate immune system, Science 327 (2010) 286-290.
287. D. Arnoult, L. Carneiro, I. Tattoli, S.E. Girardin, The role of mitochondria in cellular defense against microbial infection, Seminars in Immunology 21 (2009) 223-232.
288. M. Delgado, S. Singh, S. De Haro, S. Master, M. Ponpuak, C. Dinkins, W. Ornatowski, I. Vergne, V. Deretic, Autophagy and pattern recognition receptors in innate immunity, Immunological Reviews 227 (2009) 189-202.
289. M.C. Tal, A. Iwasaki, Autophagic control of RLR signaling, Autophagy 5 (2009) 749-750.
290. V. Deretic, Autophagy of intracellular microbes and mitochondria: two sides of the same coin? F1000 Biology Reports 2 (2010) 45.
291. C. Kohchi, H. Inagawa, T. Nishizawa, G. Soma, ROS and innate immunity, Anticancer Research 29 (2009) 817-821.
292. T. Finkel, Signal transduction by mitochondrial oxidants, Journal of Biological Chemistry 287 (2012) 4434-4440.
293. A.A. Manfredi, P. Rovere-Querini, The mitochondrion-a Trojan horse that kicks off inflammation? New England Journal of Medicine 362 (2010) 2132-2134.
294. D. Arnoult, F. Soares, I. Tattoli, S.E. Girardin, Mitochondria in innate immunity, EMBO Reports 12 (2011) 901-910.
295. A.P. West, G.S. Shadel, S. Ghosh, Mitochondria in innate immune responses, Nature Reviews Immunology 11 (2011) 389-402.
296. L. Bird, Innate immunity: linking mitochondria and microbes to inflamma-somes, Nature Reviews Immunology 12 (2012) 229.
297. S.M. Cloonan, A.M. Choi, Mitochondria: commanders of innate immunity and disease? Current Opinion in Immunology 24 (2012) 32-40.
298. O. Kepp, L. Galluzzi, G. Kroemer, Mitochondrial control of the NLRP3 inflammasome, Nature Immunology 12 (2011) 199-200.
299. J. Tschopp, Mitochondria: sovereign of inflammation? European Journal of immunology 41 (2011) 1196-1202.
300. R. Zhou, A.S. Yazdi, P. Menu, J. Tschopp, A role for mitochondria in NLRP3 inflammasome activation, Nature 469 (2011) 221-225.
301. M.S. Gelman, E.S. Kannegaard, R.R. Kopito, A principal role for the protea-some in endoplasmic reticulum-associated degradation of misfolded intra-cellular cystic fibrosis transmembrane conductance regulator, Journal of Biological Chemistry 277 (2002) 11709-11714.
302. R.J. Devenish, Autophagy and the evasion of host defense: a new variation on the theme for Burkholderia cepacia? Autophagy 7 (2011) 1269-1270.
303. A.J. Choi, S.W. Ryter, Autophagy in inflammatory diseases, International Journal of Cell Biology 2011 (2011) ID 732798.
304. J.A. Haspel, A.M. Choi, Autophagy: a core cellular process with emerging links to pulmonary disease, American Journal of Respiratory and Critical Care Medicine 184 (2011) 1237-1246.
305. S.W. Ryter, K. Nakahira, J.A. Haspel, A.M. Choi, Autophagy in pulmonary diseases, Annual Review of Physiology 74 (2012) 377-401.
306. M.A. Gavrilin, D.H. Abdelaziz, M. Mostafa, B.A. Abdulrahman, J. Grandhi, A. Akhter, A. Abu Khweek, D.F. Aubert, M.A. Valvano, M.D. Wewers, A.O. Amer, Activation of the pyrin inflammasome by intracellular Burkhol-deria cenocepacia, Journal of Immunology 188 (2012) 3469-3477.
307. A. Tang, A. Sharma, R. Jen, A.F. Hirschfeld, M.A. Chilvers, P.M. Lavoie, S.E. Turvey, Inflammasome-mediated IL-1beta production in humans with cystic fibrosis, PLoS One 7 (2012) e37689.