The cystic fibrosis transmembrane conductance regulator and ATP

Current Opinion in Cell Biology

Volumn 9, Issue 4, 1997, Pages 547-552

  Devidas, Sreenivas ^a   Guggino, William B ^a  

^a JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE TRIPHOSPHATE; CHLORIDE CHANNEL; CHLORIDE ION; PURINE RECEPTOR; SODIUM CHANNEL; SODIUM ION; TRANSMEMBRANE CONDUCTANCE REGULATOR;

CHLORIDE TRANSPORT; CYSTIC FIBROSIS; HUMAN; NUCLEOTIDE TRANSPORT; PRIORITY JOURNAL; SHORT SURVEY; SODIUM TRANSPORT;

EID: 0030860084     PISSN: 09550674     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0955-0674(97)80032-4     Document Type: Article

References (53)

1. Riordan JM, Rommens JM, Kerem BS, Alon N, Rozmahel R, Grzelvzak Z, Zeilenski J, Lok S, Plavsic N, Chou JL et al.: Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989, 245:1066-1073.
2. Anderson MP, Rich DP, Gregory RJ, Smith AE, Welsh MJ: Generation of cAMP-activated chloride currents by expression of CFTR. Science 1991, 251:679-682.
3. Bear CE, Li C, Kartner N, Bridges RJ, Jensen TJ, Ramjeesingh M, Riordan JR: Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR). Cell 1992, 68:809-818.
4. Carroll TP, McIntosh I, Egan ME, Zeitlin PL, Cutting GR, Guggino WB: Transmembrane mutations alter the channel characteristics of the cystic fibrosis transmembrane conductance regulator expressed in Xenopus oocytes. Cell Physiol Biochem 1995, 362:160-164.
5. Carson MR, Travis SM, Welsh MJ: The two nucleotide-binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) have distinct functions in controlling channel activity. J Biol Chem 1995, 270:1711-1717.
6. Cheng SH, Rich DP, Marshall J, Gregory RJ, Welsh MJ, Smith AE: Phosphorylation by the R domain by cAMP-dependent protein kinase regulates the CRR chloride channel. Cell 1991, 66:1027-1036.
7. Egan M, Flotte T, Afione S, Solow R, Zeitlin PL, Carter BJ, Guggino WB: Defective regulation of outwardly rectifying Cl-channels by protein kinase A corrected by insertion of CFTR. Nature 1992, 358:581-584.
8. Garber SS: Outwardly rectifying chloride channels in lymphocytes. J Membr Biol 1992, 127:49-56.
9. Gray MA, Harris A, Coleman L, Greenwell JR, Argent BE: Two types of chloride channels on duct cells cultured from human fetal pancreas. Am J Physiol 1989, 257:C240-C251.
10. Hwang TC, Nagel G, Nairn AC, Gadsby DC: Regulation of the gating of the cystic fibrosis transmembrane conductance regulator Cl channels by phosphorylation and ATP hydrolysis. Proc Natl Acad Sci USA 1994, 91:4698-4702.
11. Morales MM, Carroll TP, Morita T, Schwiebert EM, Devuyst O, Wilson PD, Lopes AG, Stanton BA, Dietz HC, Cutting GR, Guggino WB: Both the wild type and a functional isoform of CFTR are expressed in the kidney. Am J Physiol 1996, 270:F1038-F1048.
12. Collins FS: Cystic fibrosis: molecular biology and therapeutic implications. Science 1992, 256:774-779.
13. Cutting GR, Kasch LM, Rosenstein BJ, Zielenski J, Tsui LC, Antonarakis SE, Kazazian HHJ: A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein. Nature 1990, 346:366-369.
14. Denning GM, Ostedgaard LS, Welsh MJ: Abnormal localization of the cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia. J Cell Biol 1992, 118:551-559.
15. Gregory RJ, Rich DP, Cheng SH, Souza DW, Paul S, Manavalan P, Anderson MP, Welsh MJ, Smith AE: Maturation and function of the cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2. Mol Cell Biol 1991, 11:3886-3893.
16. Kerem BS, Zielenski J, Markiewiz D, Bozon D, Gazit E, Yahav J, Kennedy D, Riordan JR, Collins FS, Rommens JM: Identification of mutations in regions corresponding to the two putative nucleotide (ATP)-binding folds of the cystic fibrosis gene. Proc Natl Acad Sci USA 1990, 87:8447-8451.
17. Sheppard DN, Rich DP, Ostedgaard LS, Gregory RJ, Smith AE, Welsh MJ: Mutations in CFTR associated with mild disease for Cl- channels with altered pore properties. Nature 1993, 362:160-164.
18. Stutts MJ, Canessa CM, Olsen JC, Hamrick M, Cohn JA, Rossier BC, Boucher RC: CFTR as a cAMP dependent regulator of sodium channels. Science 1995, 269:847-849.
19. Gabriel SE, Clarke LL, Boucher RC, Stutts MJ: CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship. Nature 1993, 363:263-268.
20. Jovov B, Ismailov II, Benos DJ: Cystic fibrosis transmembrane conductance regulator is required for protein kinase A activation of an outwardly rectified anion channel purified from bovine tracheal epithelia. J Biol Chem 1995, 270:1521-1528,
21. 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.
22. Abraham EH, Prat AG, Gerweck L, Seneveratne T, Arceci RJ, Kramer R, Guidotti G, Cantiello HF: The multidrug resistance (mdr1) gene product functions as an ATP channel. Proc Natl Acad Sci USA 1993, 90:312-316.
23. Pasyk EA, Foskett JK: Cystic fibrosis transmembrane conductance regulator associated ATP and adenosine 3-phosphate 5-phosphosulfate channels in the endoplasmic reticulum and plasma membranes. J Biol Chem 1996, 272:7746-7751. A recent study demonstrating that ATP can be transported through the CFTR. Using the patch clamp technique, the authors demonstrate ATP transport in select clones of IB3 cells, both at the endoplasmic reticulum and at the plasma membrane.
24. Reddy MM, Quinton PM, Haws C, Wine JJ, Grygorczyk R, Tabcharani JA, Hanrahan JW, Gunderson KL, Kopito RR: Failure of the cystic fibrosis transmembrane conductance regulator to conduct ATP. Science 1996, 271:1876-1879. A rebuttal of the controversial relationship between the CFTR and ATP. This group of investigators did not observe ATP transport through the CFTR in a variety of cell types.
25. Grygorczyk R, Tabcharani JA, Hanrahan JW: CFTR channels expressed in CHO cells do not have detectable ATP conductance. J Membr Biol 1996, 151:139-148. This study of ATP transport in Chinese hamster ovary cells expressing the CFTR reveals that there is no detectable ATP transport through the CFTR.
26. Canhui L, Mohabir R, Bear CE: Purified cystic fibrosis transmembrane conductance regulator (CFTR) does not function as an ATP channel. J Biol Chem 1995, 271:11623-11626.
27. Schwiebert EM, Egan ME, Hwang TH, Fulmer SB, Allen SS, Cutting GR, Guggino WB: CFTR regulates outwardly rectifying chloride currents through an autocrine mechanism involving ATP. Cell 1995, 81:1063-1073.
28. Abraham EH, Okunieff P, Scala S, Vos P, Oosterveld MJS, Chen AY, Shrivastav B: Cystic fibrosis transmembrane conductance regulator and adenosine triphosphate. Science 1997, 275:1324-1325. A luminometry study showing that the CFTR conducts, or is associated with the transport of, ATP out of cells. Use of ectoATPases resulted in a further increase in ATP released from cells transfected with the CFTR as compared with mock transfected cells.
29. Abraham EH, Vos P, Kahn J, Grubman SA, Jefferson DM, Ding I, Okunieff P: Cystic fibrosis hetero- and homozygosity is associated with inhibition of breast cancer growth. Nat Med 1996, 2:593-596.
30. Pasyk EA, Foskett JK: Mutant (delta F508) cystic fibrosis transmembrane conductance regulator Cl- channel is functional when retained in endoplasmic reticulum of mammalian cells. J Biol Chem 1995, 270:12347-12350.
31. Cheng PW, Boat TF, Cranfill K, Yankaskas JR, Boucher RC: Increased sulfation of glycoconjugates by cultured nasal epithelial cells from patients with cystic fibrosis. J Clin Invest 1989, 84:68-72.
32. Mohapatra NK, Cheng PW, Parker JC, Paradiso AM, Yankaskas JR, Boucher RC, Boat TF: Alteration of sulfation of glycoconjugates, but not sulfate transport and intracellular inorganic sulfate content in cystic fibrosis airway epithelial cells. Pediatr Res 1995, 38:42-48.
33. ••], demonstrating a failure to detect significant ATP release in cells transfected with the CFTR. The only release of ATP observed was associated with mechanical stretching of cells on a flexible substrate.
34. Arispe N, Rojas E, Hartman J, Sorscher EJ, Pollard HB: Intrinsic anion channel activity of the recombinant first nucleotide binding fold domain of the cystic fibrosis transmembrane regulator. Proc Natl Acad Sci USA 1992, 89:1539-1543.
35. Sheng M: PDZs and receptor/channel clustering: rounding up the latest suspects. Neuron 1996, 17:575-578. An excellent review summarizing recent findings about channel/receptor clustering at the plasma membrane and the proteins associated with such clustering. This has important consequences in understanding the mechanisms of ion channel interactions at the plasma membrane.
36. + channels by interaction with a family of membrane-associated guanylate kinases. Nature 1995, 378:85-88.
37. Kim E, Cho KO, Rothschild A, Sheng M: Heteromultimerization and NMDA receptor-clustering activity of Chapsyn-110, a member of the PSD-95 family of proteins. Neuron 1996, 17:103-113.
38. Anderson MP, Berger HA, Rich DP, Gregory RJ, Smith AE, Welsh MJ: Nucleotide triphosphates are required to open the CFTR chloride channel. Cell 1991, 87:775-784.
39. Cheng SH, Gregory RJ, Marshall J, Paul S, Souza DW, White GA, O'Riordan CR, Smith AE: Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 1990, 63:827-834.
40. Hwang TC, Lu L, Zeitlin PL, Gruenert DC, Huganir R, Guggino WB: Chloride channels in CF: lack of activation by protein kinase C and cAMP-dependent protein kinase. Science 1989, 244:1351-1353.
41. Knowles M, Gatzy J, Boucher R: Relative ion permeability of normal and cystic fibrosis nasal epithelium. J Clin Invest 1983, 71:1410-1417.
42. Picciotto MR, Cohn JA, Bertuzzi G, Greengard P, Nairn AC: Phosphorylation of cystic fibrosis transmembrane conductance regulator. J Biol Chem 1992, 267:12742-12752.
43. Tabcharani JA, Chang XB, Riordan JR, Hanrahan JW: The cystic fibrosis transmembrane conductance regulator chloride channel. Iodide block and permeation. Biophys J 1992, 62:1-4.
44. Tabcharani JA, Chang XB, Riordan JR, Hanrahan JW: Phosphorylation-regulated Cl- channel in CHO cells stably expressing the cystic fibrosis gene. Nature 1991, 352:628-631.
45. Widdicombe JH, Welsh MJ, Finkbeiner WE: Cystic fibrosis decreases the apical membrane chloride permeability of monolayers cultured from cells of tracheal epithelium. Proc Natl Acad Sci USA 1985, 82:6167-6171.
46. 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 1991, 88:7500-7504.
47. Widdicombe JH, Widdicombe JG: Regulation of human airway surface liquid. Respir Physiol 1995, 99:3-12.
48. + transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. J Clin Invest 1986, 78:1245-1252.
49. Ames GFL, Mimura CS, Shyamala V: Bacterial periplasmic permeases belong to a family of transport proteins operating from Escherichia coli to human traffic ATPases. FEMS Microbiol Res 1990, 75:429-446.
50. Deversen EV, Gow IR, Coadwell WJ, Monaco JJ, Butcher GW, Howard JC: MHC class Il region encoding proteins related to the multidrug resistance family of transmembrane transporters. Nature 1990, 348:738-741.
51. Higgins CF, Hiles ID, Salmond GPC, Gill DR, Downie JA, Evans IJ, Holland IB, Buckel SD, Bell AW, Hermondson MA: A family of related ATP-binding subunits coupled to many distinct biological processes in bacteria. Nature 1986, 323:448-450.
52. Hyde SC, Emsley P, Hartshorn MJ, Mimmack MM, Gileadi U, Pearce SR, Gallagher MP, Gill DR, Hubbard RE, Higgins CF: Structural model of ATP binding proteins associated with cystic fibrosis, multidrug resistance and bacterial transport. Nature 1990, 346:362-365.
53. Walker JE, Saraste M, Runswick MJ, Gay NJ: Distantly related sequences in the α and β subunits of ATP synthase, myosin, kinases and other ATP requiring enzymes and a common nucleotide binding fold. EMBO J 1982, 1:945-951.