cAMP-mediated regulation of cholesterol accumulation in cystic fibrosis and Niemann-Pick type C cells

American Journal of Physiology - Lung Cellular and Molecular Physiology

Volumn 295, Issue 5, 2008, Pages

  Manson, Mary E ^a   Corey, Deborah A ^a   White, Nicole M ^a   Kelley, Thomas J ^a,b  

^a CASE WESTERN RESERVE UNIVERSITY   (United States)

^b CASE WESTERN RESERVE UNIVERSITY   (United States)

Author keywords

Rp diastereomer of adenosine 3 ,5 cyclic monophosphothioate

Indexed keywords

8 BROMO CYCLIC AMP; ADENOSINE 3',5' PHOSPHOROTHIOATE; BETA 2 ADRENERGIC RECEPTOR; BETA ARRESTIN 2; CHOLESTEROL; CYCLIC AMP; FILIPIN; G PROTEIN COUPLED RECEPTOR KINASE 2; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ARTICLE; CELL CULTURE; CELL LINE; CONTROLLED STUDY; CYSTIC FIBROSIS; DIASTEREOISOMER; EPITHELIUM CELL; FEEDBACK SYSTEM; HUMAN; HUMAN CELL; LUNG FIBROBLAST; MOUSE; NIEMANN PICK DISEASE; NONHUMAN; NORMAL HUMAN; NOSE MUCOSA; PRIORITY JOURNAL; PROTEIN EXPRESSION; SIGNAL TRANSDUCTION; WILD TYPE;

8-BROMO CYCLIC ADENOSINE MONOPHOSPHATE; ANIMALS; ARRESTINS; CELL LINE; CHOLESTEROL; CYCLIC AMP; CYCLIC AMP RESPONSE ELEMENT-BINDING PROTEIN; CYSTIC FIBROSIS; EPITHELIUM; FIBROBLASTS; HUMANS; MICE; NIEMANN-PICK DISEASE, TYPE C; PHOSPHORYLATION; PROTEIN TRANSPORT; RECEPTORS, ADRENERGIC, BETA-2; THIONUCLEOTIDES;

EID: 57049085932     PISSN: 10400605     EISSN: 15221504     Source Type: Journal    
DOI: 10.1152/ajplung.90402.2008     Document Type: Article

References (45)

1. Armstrong DS, Grimwood K, Carlin JB, Carzino R, Gutièrrez JP, Hull J, Olinsky A, Phelan EM, Robertson CF, Phelan PD. Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med 156: 1197-1204, 1997.
2. Armstrong DS, Hook SM, Jamsen KM, Nixon GM, Carzino R, Carlin JB, Robertson CF, Grimwood K. Lower airway inflammation in infants with cystic fibrosis detected by newborn screening. Pediatr Pulmonol 40: 500-510, 2005.
3. Barnes AP, Livera G, Huang P, Sun C, O'Neal WK, Conti M, Stutts MJ, Milgram SL. Phosphodiesterase 4D forms a cAMP diffusion barrier at the apical membrane of the airway epithelium. J Biol Chem 280: 7997-8003, 2005.
4. Becker MN, Sauer MS, Muhlebach MS, Hirsh AJ, Wu Q, Verghese MW, Randell SH. Cytokine secretion by cystic fibrosis airway epithelial cells. Am J Respir Crit Care Med 169: 645-653, 2004.
5. Boncoeur E, Criq VS, Bonvin E, Roque T, Henrion-Caude A, Gruenert DC, Clement A, Jacquot J, Tabary O. Oxidative stress induces extracellular signal-regulated kinase 1/2 mitogen-activated protein kinase in cystic fibrosis lung epithelial cells: potential mechanism for excessive IL-8 expression. Int J Biochem Cell Biol 40: 432-446, 2008.
6. DiMango E, Ratner AJ, Bryan R, Tabibi S, Prince A. Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells. J Clin Invest 101: 2598-2605, 1998.
7. Dötsch J, Puls J, Klimek T, Rascher W. Reduction of neuronal and inducible nitric oxide synthase gene expression in patients with cystic fibrosis. Eur Arch Otorhinolaryngol 259: 222-226, 2002.
8. Eckman EA, Cotton CU, Kube DM, Davis PB. Dietary changes improve survival of CFTR S489X homozygous mutant mouse. Am J Physiol Lung Cell Mol Physiol 269: L625-L630, 1995.
9. Ferguson SS, Zhang J, Barak LS, Caron MG. Molecular mechanisms of G protein-coupled receptor desensitization and resensitization. Life Sci 62: 1561-1565, 1998.
10. Gentzsch M, Choudhury A, Chang XB, Pagano RE, Riordan JR. Misassembled mutant ΔF508 CFTR in the distal secretory pathway alters cellular lipid trafficking. J Cell Sci 120: 447-455, 2007.
11. Gévry NY, Lalli E, Sassone-Corsi P, Murphy BD. Regulation of Niemann-Pick C1 gene expression by the 3′,5′-cyclic adenosine monophosphate pathway in steroidogenic cells. Mol Endocrinol 17: 704-715, 2003.
12. Hajj R, Lesimple P, Nawrocki-Raby B, Birembaut P, Puchelle E, Coraux C. Human airway surface epithelial regeneration is delayed and abnormal in cystic fibrosis. J Pathol 211: 340-350, 2007.
13. Heeckeren A, Walenga R, Konstan MW, Bonfield T, Davis PB, Ferkol T. Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa. J Clin Invest 100: 2810-2815, 1997.
14. 2+ of airway epithelia. Am J Physiol Lung Cell Mol Physiol 293: L1250-L1260, 2007.
15. Kelley TJ, Drumm ML. Inducible nitric oxide synthase expression is reduced in cystic fibrosis murine and human airway epithelial cells. J Clin Invest 102: 1200-1207, 1998.
16. Kelley TJ, Elmer HL. In vivo alterations of IFN regulatory factor-1 and PIAS1 protein levels in cystic fibrosis epithelium. J Clin Invest 106: 403-410, 2000.
17. Konstan MW, Davis PB, Wagener JS, Hilliard KA, Stern RC, Milgram LJ, Kowalczyk TH, Hyatt SL, Fink TL, Gedeon CR, Oette SM, Payne JM, Muhammad O, Ziady AG, Moen RC, Cooper MJ. Compacted DNA nanoparticles administered to the nasal mucosa of cystic fibrosis subjects are safe and demonstrate partial to complete cystic fibrosis transmembrane regulator reconstitution. Hum Gene Ther 15: 1255-1269, 2004.
18. Kreiselmeier NE, Kraynack NC, Corey DA, Kelley TJ. Statin-mediated correction of STAT1 signaling and inducible nitric oxide synthase expression in cystic fibrosis epithelial cells. Am J Physiol Lung Cell Mol Physiol 285: L1286-L1295, 2003.
19. Kruth HS, Comly ME, Butler JD, Vanier MT, Fink JK, Wenger DA, Patel S, Pentchev PG. Type C Niemann-Pick disease. Abnormal metabolism of low density lipoprotein in homozygous and heterozygous fibroblasts. J Biol Chem 261: 16769-16774, 1986.
20. Kube D, Sontich U, Fletcher D, Davis PB. Proinflammatory cytokine responses to P. aeruginosa infection in human airway epithelial cell lines. Am J Physiol Lung Cell Mol Physiol 280: L493-L502, 2001.
21. Lemberger T, Parkitna JR, Chai M, Schütz G, Engblom D. CREB has a context-dependent role in activity-regulated transcription and maintains neuronal cholesterol homeostasis. FASEB J 22: 2872-2879, 2008.
22. Mak JC, Chuang TT, Harris CA, Barnes PJ. Increased expression of G protein-coupled receptor kinases in cystic fibrosis lung. Eur J Pharmacol 436: 165-172, 2002.
23. Matthews RP, McKnight GS. Characterization of the cAMP response element of the cystic fibrosis transmembrane conductance regulator gene promoter. J Biol Chem 271: 31869-31877, 1996.
24. Meng QH, Polak JM, Edgar AJ, Chacon MR, Evans TJ, Gruenert DC, Bishop AE. Neutrophils enhance expression of inducible nitric oxide synthase in human normal but not cystic fibrosis bronchial epithelial cells. J Pathol 190: 126-132, 2000.
25. Meng QH, Springall DR, Bishop AE, Morgan K, Evans TJ, Habib S, Gruenert DC, Gyi KM, Hodson ME, Yacoub MH, Polak JM. Lack of inducible nitric oxide synthase in bronchial epithelium: a possible mechanism of susceptibility to infection in cystic fibrosis. J Pathol 184: 323-331, 1998.
26. Moore RH, Tuffaha A, Millman EE, Dai W, Hall HS, Dickey BF, Knoll BJ. Agonist-induced sorting of human beta2-adrenergic receptors to lysosomes during downregulation. J Cell Sci 112: 329-338, 1999.
27. Morrissey BM, Schilling K, Weil JV, Silkoff PE, Rodman DM. Nitric oxide and protein nitration in the cystic fibrosis airway. Arch Biochem Biophys 406: 33-39, 2002.
28. Perez A, Issler AC, Cotton CU, Kelley TJ, Verkman AS, Davis PB. CFTR inhibition mimics the cystic fibrosis inflammatory profile. Am J Physiol Lung Cell Mol Physiol 292: L383-L395, 2007.
29. - secretion in 9/HTEo- cells in culture. Am J Physiol Lung Cell Mol Physiol 271: L85-L92, 1996.
30. Poschet JF, Fazio JA, Timmins GS, Ornatowski W, Perkett E, Delgado M, Deretic V. Endosomal hyperacidification in cystic fibrosis is due to defective nitric oxide-cyclic GMP signalling cascade. EMBO Rep 7: 553-559, 2006.
31. Rommens JM, Dho S, Bear CE, Kartner N, Kennedy D, Riordan JR, Tsui LC, Foskett JK. cAMP-inducible chloride conductance in mouse fibroblast lines stably expressing the human cystic fibrosis transmembrane conductance regulator. Proc Natl Acad Sci USA 88: 7500-7504, 1991.
32. Scott C, Higgins ME, Davies JP, Ioannou YA. Targeting of NPC1 to late endosomes involves multiple signals, including one residing within the putative sterol-sensing domain. J Biol Chem 279: 48214-48223, 2004.
33. Sharma RK, Jeffery PK. Airway beta-adrenoceptor number in cystic fibrosis and asthma. Clin Sci (Lond) 78: 409-417, 1990.
34. Srivastava M, Eidelman O, Zhang J, Paweletz C, Caohuy H, Yang Q, Jacobson KA, Heldman E, Huang W, Jozwik C, Pollard BS, Pollard HB. Digitoxin mimics gene therapy with CFTR and suppresses hypersecretion of IL-8 from cystic fibrosis lung epithelial cells. Proc Natl Acad Sci USA 101: 7693-7698, 2004.
35. Teichgräber V, Ulrich M, Endlich N, Riethmüller J, Wilker B, De Oliveira-Munding CC, van Heeckeren AM, Barr ML, von Kürthy G, Schmid KW, Weller M, Tumbler B, Lang F, Grasse H, During G, Goblins E. Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis. Nat Med 14: 382-391, 2008.
36. Thomas GR, Costelloe EA, Lunn DP, Stacey KJ, Delaney SJ, Passey R, McGlinn EC, McMorran BJ, Ahadizadeh A, Geczy CL, Wainwright BJ, Hume DA. G551D cystic fibrosis mice exhibit abnormal regulation of inflammation in lungs and macrophages. J Immunol 164: 3870-3877, 2000.
37. Tirouvanziam R, de Bentzmann S, Hubeau C, Hinnrasky J, Jacquot J, Péault, B Puchelle E. Inflammation and infection in naive human cystic fibrosis airway grafts. Am J Respir Cell Mol Biol 23: 121-127, 2000.
38. Van Heeckeren AM, Schluchter MD, Drumm ML, Davis PB. Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice. Am J Physiol Lung Cell Mol Physiol 287: L944-L952, 2004.
39. - channel function. Am J Physiol Lung Cell Mol Physiol 281: L71-L78, 2001.
40. White NM, Corey DA, Kelley TJ. Mechanistic similarities between cultured cell models of cystic fibrosis and Niemann-Pick type C. Am J Respir Cell Mol Biol 31: 538-543, 2004.
41. White NM, Jiang D, Burgess JD, Bederman IR, Previs SF, Kelley TJ. Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis. Am J Physiol Lung Cell Mol Physiol 292: L476-L486, 2007.
42. Widdicombe JH. Cystic fibrosis and β-adrenergic response of airway epithelial cell cultures. Am J Physiol Regul Integr Comp Physiol 251: R818-R822, 1986.
43. Wu JH, Peppel K, Nelson CD, Lin FT, Kohout TA, Miller WE, Exum ST, Freedman NJ. The adaptor protein beta-arrestin2 enhances endocytosis of the low density lipoprotein receptor. J Biol Chem 278: 44238-44245, 2003.
44. Xu W, Zheng S, Goggans TM, Kiser P, Quinones-Mateu ME, Janocha AJ, Comhair SA, Slee R, Williams BR, Erzurum SC. Cystic fibrosis and normal human airway epithelial cell response to influenza a viral infection. J Interferon Cytokine Res 26: 609-627, 2006.
45. Zheng S, Xu W, Bose S, Banerjee AK, Haque SJ, Erzurum SC. Impaired nitric oxide synthase-2 signaling pathway in cystic fibrosis airway epithelium. Am J Physiol Lung Cell Mol Physiol 287: L374-L381, 2004.