Expression of delta F508 cystic fibrosis transmembrane conductance regulator protein and related chloride transport properties in the gallbladder epithelium from cystic fibrosis patients

Hepatology

Volumn 29, Issue 6, 1999, Pages 1624-1634

  Dray Charier, N ^a   Paul, A ^a   Scoazec, J Y ^a   Veissiere, D ^a   Mergey, M ^a   Capeau, J ^a   Soubrane, O ^a   Housset, C ^a  

^a INSERM   (France)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE CHANNEL; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ADOLESCENT; ADULT; ARTICLE; CHLORIDE TRANSPORT; CYSTIC FIBROSIS; FEMALE; GALLBLADDER EPITHELIUM; HUMAN; HUMAN TISSUE; MALE; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN LOCALIZATION;

EID: 0033036472     PISSN: 02709139     EISSN: None     Source Type: Journal    
DOI: 10.1002/hep.510290634     Document Type: Article

References (57)

1. 1 Riordan JR, Rommens JM, Kerem B‐s, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989 ; 245: 1066–1073. MEDLINE
2. 2 Mack DR, Traystman MD, Colombo JL, Sammut PH, Kaufman SS, Vanderhoof JA, Antonson DL, et al. Clinical denouement and mutation analysis of patients with cystic fibrosis undergoing liver transplantation for biliary cirrhosis. J Pediatr 1995 ; 127: 881–887. MEDLINE
3. 3 Hultcrantz R, Mengarelli S, Strandvik B. Morphological findings in the liver of children with cystic fibrosis. A light and electron microscopical study. Hepatology 1986 ; 6: 881–889. MEDLINE
4. 4 Gaskin KJ, Waters DLM, Howman‐Giles R, de Silva M, Earl JW, Martin HCO, Kan AE, et al. Liver disease and common‐bile‐duct stenosis in cystic fibrosis. N Engl J Med 1988 ; 318: 340–346. MEDLINE
5. 5 Lindblad A, Hultcrantz R, Strandvik B. Bile‐duct destruction and collagen deposition: a prominent ultrastructural feature of the liver in cystic fibrosis. Hepatology 1992 ; 16: 372–381. MEDLINE
6. 6 Waters DL, Dorney SFA, Gruca MA, Martin HCO, Howman‐Giles R, Kan AE, De Silva M, et al. Hepatobiliary disease in cystic fibrosis patients with pancreatic sufficiency. Hepatology 1995 ; 21: 963–969. MEDLINE
7. 7 Jebbink MCW, Heijerman HGM, Masclee AAM, Lamers CBHW. Gallbladder disease in cystic fibrosis. Neth J Med 1992 ; 41: 123–126. MEDLINE
8. 8 Anderson MP, Gregory RJ, Thompson S, Souza DW, Paul S, Mulligan RC, Smith AE, et al. Demonstration that CFTR is a chloride channel by alteration of its anion selectivity. Science 1991 ; 253: 202–205. MEDLINE
9. 9 Cohn JA, Strong TV, Picciotto MR, Nairn AC, Collins FS, Fitz JG. Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells. Gastroenterology 1993 ; 105: 1857–1864. MEDLINE
10. 10 Kerem B‐s, Rommens JM, Buchanan JA, Markiewicz D, Cox TK, Chakravarti A, Buchwald M, et al. Identification of the cystic fibrosis gene; genetic analysis. Science 1989 ; 245: 1073–1080. MEDLINE
11. 11 Cheng SH, Gregory RJ, Marshall J, Paul S, Souza DW, White GA, O'Riordan CR, et al. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 1990 ; 63: 827–834. MEDLINE
12. 12 Dalemans W, Hinnrasky J, Slos P, Dreyer D, Fuchey C, Pavirani A, Puchelle E. Immunocytochemical analysis reveals differences between the subcellular localization of normal and ΔPhe508 recombinant cystic fibrosis transmembrane conductance regulator. Exp Cell Res 1992 ; 201: 235–240. MEDLINE
13. 13 Oppenheimer EH, Esterly JR. Hepatic changes in young infants with cystic fibrosis: possible relation to focal biliary cirrhosis. J Pediatr 1975 ; 86: 683–689. MEDLINE
14. 14 Boyer JL. Bile duct epithelium: frontiers in transport physiology. Am J Physiol 1996 ; 270: G1–G5. MEDLINE
15. 15 Clarke LL, Grubb BR, Yankaskas JR, Cotton CU, McKenzie A, Boucher RC. Relationship of a non‐cystic fibrosis transmembrane conductance regulator–mediated chloride conductance to organ‐level disease in Cftr (−/−) mice. Proc Natl Acad Sci U S A 1994 ; 91: 479–483. MEDLINE
16. 16 Schlenker T, Romac JM, Sharara AI, Roman RM, Kim SJ, LaRusso N, Liddle RA, et al. Regulation of biliary secretion through apical purinergic receptors in cultured rat cholangiocytes. Am J Physiol 1997 ; 273: G1108–G1117. MEDLINE
17. 17 Boarder MR, Hourani SMO. The regulation of vascular function by P2 receptors: multiple sites and multiple receptors. Trends Pharmacol Sci 1998 ; 19: 99–107. MEDLINE
18. 18 Dray‐Charier N, Paul A, Veissière D, Mergey M, Scoazec J‐Y, Capeau J, Brahimi‐Horn C, et al. Expression of cystic fibrosis transmembrane conductance regulator in human gallbladder epithelial cells. Lab Invest 1995 ; 73: 828–836. MEDLINE
19. 19 Chinet T, Fouassier L, Dray‐Charier N, Imam‐Ghali M, Morel H, Mergey M, Dousset B, et al. Regulation of electrogenic anion secretion in normal and cystic fibrosis gallbladder mucosa. Hepatology 1999 ; 29: 5–13. MEDLINE
20. 20 Scoazec J‐Y, Bringuier A‐F, Medina JF, Martinez‐Anso E, Veissière D, Feldmann G, Housset C. The plasma membrane polarity of human biliary epithelial cells: in situ immunohistochemical analysis and functional implications. J Hepatol 1997 ; 26: 543–553. MEDLINE
21. 21 Graham RC, Karnovsky MJ. The early stages of absorption of horseradish peroxidase in the proximal tubules of mouse kidney: ultrastructural cytochemistry by a new technique. J Histochem Cytochem 1966 ; 14: 291–302. MEDLINE
22. 22 Housset C, Carayon A, Housset B, Legendre C, Hannoun L, Poupon R. Endothelin‐1 secretion by human gallbladder epithelial cells in primary culture. Lab Invest 1993 ; 69: 750–755. MEDLINE
23. 23 Yankaskas JR, Cotton CU, Knowles MR, Gatzy JT, Boucher RC. Culture of human nasal epithelial cells on collagen matrix supports. Am Rev Respir Dis 1985 ; 132: 1281–1287. MEDLINE
24. 24 Marshall J, Fang S, Ostedgaard LS, O'Riordan CR, Ferrara D, Amara JF, Hoppe IV H, et al. Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro. J Biol Chem 1994 ; 269: 2987–2995. MEDLINE
25. 25 Sheppard DN, Ostedgaard LS, Rich DP, Welsh MJ. The amino‐terminal portion of CFTR forms a regulated Cl − channel. Cell 1994 ; 76: 1091–1098. MEDLINE
26. 26 Zeitlin PL, Crawford I, Lu L, Woel S, Cohen ME, Donowitz M, Montrose MH, et al. CFTR protein expression in primary and cultured epithelia. Proc Natl Acad Sci U S A 1992 ; 89: 344–347. MEDLINE
27. 27 Cheng SH, Fang SL, Zabner J, Marshall J, Piraino S, Schiavi SC, Jefferson DM, et al. Functional activation of the cystic fibrosis trafficking mutant ΔF508‐CFTR by overexpression. Am J Physiol 1995 ; 268: L615–L624. MEDLINE
28. 28 Yang Y, Devor DC, Engelhardt JF, Ernst SA, Strong TV, Collins FS, Cohn JA, et al. Molecular basis of defective anion transport in L cells expressing recombinant forms of CFTR. Hum Mol Genet 1993 ; 2: 1253–1261. MEDLINE
29. 29 Kartner N, Augustinas O, Jensen TJ, Naismith AL, Riordan JR. Mislocalization of ΔF508 CFTR in cystic fibrosis sweat gland. Nat Genet 1992 ; 1: 321–327. MEDLINE
30. 30 Puchelle E, Gaillard D, Ploton D, Hinnrasky J, Fuchey C, Boutterin M‐C, Jacquot J, et al. Differential localization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis airway epithelium. Am J Respir Cell Mol Biol 1992 ; 7: 485–491. MEDLINE
31. 31 O'Loughlin EV, Hunt DM, Bostrom TE, Hunter D, Gaskin KJ, Gyory A, Cockayne DJH. X‐ray microanalysis of cell elements in normal and cystic fibrosis jejunum: Evidence for choloride secretion in villi. Gastroenterology 1996 ; 110: 411–418. MEDLINE
32. 32 Sarkadi B, Bauzon D, Huckle WR, Earp HS, Berry A, Suchindran H, Price EM, et al. Biochemical characterization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis epithelial cells. J Biol Chem 1992 ; 267: 2087–2095. MEDLINE
33. 33 Dupuit F, Kälin N, Brézillon S, Hinnrasky J, Tümmler B, Puchelle E. CFTR and differentiation markers expression in non‐CF and ΔF 508 homozygous CF nasal epithelium. J Clin Invest 1995 ; 96: 1601–1611. MEDLINE
34. 34 Wei X, Eisman R, Xu J, Harsch AD, Mulberg AE, Bevins CL, Glick MC, et al. Turnover of the cystic fibrosis transmembrane conductance regulator (CFTR): slow degradation of wild‐type and ΔF508 CFTR in surface membrane preparations of immortalized airway epithelial cells. J Cell Physiol 1996 ; 168: 373–384. MEDLINE
35. 35 Denning GM, Anderson MP, Amara JF, Marshall J, Smith AE, Welsh MJ. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature‐sensitive. Nature 1992 ; 358: 761–764. MEDLINE
36. 36 Sato S, Ward CL, Krouse ME, Wine JJ, Kopito RR. Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation. J Biol Chem 1996 ; 271: 635–638. MEDLINE
37. 37 Teem JL, Berger HA, Ostedgaard LS, Rich DP, Tsui L‐C, Welsh MJ. Identification of revertants for the cystic fibrosis ΔF508 mutation using STE6‐CFTR chimeras in yeast. Cell 1993 ; 73: 335–346. MEDLINE
38. 38 Haws CM, Nepomuceno IB, Krouse ME, Wakelee H, Law T, Xia Y, Nguyen H, et al. ΔF508‐CFTR channels: kinetics, activation by forskolin, and potentiation by xanthines. Am J Physiol 1996 ; 270: C1544–C1555. MEDLINE
39. 39 Brezillon S, Dupuit F, Hinnrasky J, Marchand V, Kalin N, Tümmler B, Puchelle E. Decreased expression of the CFTR protein in remodeled human nasal epithelium from non‐cystic fibrosis patients. Lab Invest 1995 ; 72: 191–200. MEDLINE
40. 40 Dalemans W, Barbry P, Champigny G, Jallat S, Dott K, Dreyer D, Crystal RG, et al. Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation. Nature 1991 ; 354: 526–528. MEDLINE
41. 41 Drumm ML, Wilkinson DJ, Smit LS, Worrell RT, Strong TV, Frizzel RA, Dawson DC, et al. Chloride conductance expressed by ΔF508 and other mutant CFTRs in Xenopus oocytes. Science 1991 ; 254: 1797–1799. MEDLINE
42. 42 Igimi H, Yamamoto F, Lee SP. Gallbladder mucosal function: studies in absorption and secretion in humans and in dog gallbladder epithelium. Am J Physiol 1992 ; 263: G69–G74. MEDLINE
43. 43 Peters RHPC, van Doorninck JH, French PJ, Ratcliff R, Evans MJ, Colledge WH, Bijman J, et al. Cystic fibrosis transmembrane conductance regulator mediates the cyclic adenosine monophosphate‐induced fluid secretion but not the inhibition of resorption in mouse gallbladder epithelium. Hepatology 1997 ; 25: 270–277. MEDLINE
44. 44 Knowles MR, Clarke LL, Boucher RC. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N Engl J Med 1991 ; 325: 533–538. MEDLINE
45. 45 Tirouvanziam R, Desternes M, Saari A, Puchelle E, Péault B, Chinet T. Bioelectric properties of human cystic fibrosis and non‐cystic fibrosis fetal tracheal xenografts in SCID mice. Am J Physiol 1998 ; 274: C875–C882. MEDLINE
46. 46 McGill JM, Basavappa S, Mangel AW, Shimokura GH, Middleton JP, Fitz JG. Adenosine triphosphate activates ion permeabilities in biliary epithelial cells. Gastroenterology 1994 ; 107: 236–243. MEDLINE
47. 47 McGill JM, Yen MS, Basavappa S, Mangel AW, Kwiatkowski AP. ATP‐activated chloride permeability in biliary epithelial cells is regulated by calmodulin‐dependent protein kinase II. Biochem Biophys Res Commun 1995 ; 208: 457–462. MEDLINE
48. 48 Wolkoff LI, Perrone RD, Grubman SA, Lee DW, Soltoff SP, Rogers LC, Beinborn M, et al. Purinoreceptors P 2U identification and function in human intrahepatic biliary epithelial cell lines. Cell Calcium 1995 ; 17: 375–383. MEDLINE
49. 49 Grubb BR, Vick RN, Boucher RC. Hyperabsorption of Na + and raised Ca 2+ ‐mediated Cl − secretion in nasal epithelia of CF mice. Am J Physiol 1994 ; 266: C1478–C1483. MEDLINE
50. 50 Johnson LG, Boyles SE, Wilson J, Boucher RC. Normalization of raised sodium absorption and raised calcium‐mediated chloride secretion by adenovirus‐mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway cells. J Clin Invest 1995 ; 95: 1377–1382. MEDLINE
51. 51 Kunzelmann K, Mall M, Briel M, Hipper A, Nitschke R, Ricken S, Greger R. The cystic fibrosis transmembrane conductance regulator attenuates the endogenous Ca 2+ activated Cl − conductance of Xenopus oocytes. Pflugers Arch Eur J Physiol 1997 ; 35: 178–181.
52. 52 Veeze HJ, Halley DJJ, Bijman J, de Jongste JC, de Jonge HR, Sinaasappel M. Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype. J Clin Invest 1994 ; 93: 461–466. MEDLINE
53. 53 Reisin IL, Prat AG, Abraham EH, Amara JF, Gregory RJ, Ausiello DA, Cantiello HF. The cystic fibrosis transmembrane conductance regulator is a dual ATP and chloride channel. J Biol Chem 1994 ; 269: 20584–20591. MEDLINE
54. 54 Chari RS, Schutz SM, Haebig JE, Shimokura GH, Cotton PB, Fitz JG, Meyers WC. Adenosine nucleotides in bile. Am J Physiol 1996 ; 270: G246–G252. MEDLINE
55. 55 Sasaki H, Schaffner F, Popper H. Bile ductules in cholestasis: Morphologic evidence for secretion and absorption in man. Lab Invest 1967 ; 16: 84–95. MEDLINE
56. 56 Tavoloni N. Role of ductular bile water reabsorption in canine bile secretion. J Lab Clin Med 1985 ; 106: 154–161. MEDLINE
57. 57 Alpini G, Lenzi R, Sarkosi L, Tavoloni N. Biliary physiology in rats with bile ductular cell hyperplasia. Evidence for a secretory function of proliferated bile ductules. J Clin Invest 1988 ; 81: 569–578. MEDLINE