colforsin has been researched along with Cystic Fibrosis in 170 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 3 (1.76) | 18.7374 |
| 1990's | 77 (45.29) | 18.2507 |
| 2000's | 49 (28.82) | 29.6817 |
| 2010's | 28 (16.47) | 24.3611 |
| 2020's | 13 (7.65) | 2.80 |
| Authors | Studies |
|---|---|
| Balch, WE; Frizzell, RA; Gentzsch, M; Gottesfeld, JM; Herman, D; Hoch, B; Hutt, DM; Kellner, W; Kelly, JW; Lukacs, GL; Manning, G; Matsumura, Y; Matteson, J; Noel, S; Okiyoneda, T; Pilewski, JM; Riordan, JR; Rodrigues, AP; Schmidt, A; Skach, WR; Sorscher, EJ; Thomas, PJ; Yates, JR | 1 |
| Cho, HJ; Choi, JY; Engelhardt, JF; Joo, NS; Milla, CE; Shinbashi, M; Wine, JJ | 1 |
| Beazley, S; Burmester, M; Campanucci, VA; Carmalt, JL; Duke, T; Fujiyama, M; Gray, B; Ianowski, JP; Jagadeeshan, S; Le, Y; Luan, X; Machen, TE; Murray, B; Shipley, A; Swekla, K; Tam, JS | 1 |
| Beekman, JM; Berkers, G; Brunsveld, JE; de Poel, E; de Winter-de Groot, KM; Dompeling, E; Eijkemans, MJC; Hagemeijer, MC; Heida-Michel, S; Jans, SR; Koppelman, GH; Kruisselbrink, E; Muilwijk, D; Oppelaar, H; Roukema, J; Suen, SWF; van der Eerden, MM; van der Ent, CK; van der Meer, R; van Mourik, P; van Panhuis, H; Vonk, AM; Vries, R; Weersink, EJM; Zomer-van Ommen, DD | 1 |
| Aalbers, BL; Beekman, JM; Brunsveld, JE; Heijerman, HGM; van den Eijnden, JC; van der Ent, CK | 1 |
| Adyan, T; Bukharova, T; Bulatenko, N; Efremova, A; Goldshtein, D; Kondratyeva, E; Kovalskaia, V; Kutsev, S; Melyanovskaya, Y; Petrova, N; Polyakov, A; Sherman, V; Starinova, M; Voronkova, A | 1 |
| Bertini, M; Conti, J; Farinazzo, A; Kleinfelder, K; Latorre, RV; Lotti, V; Massella, A; Melotti, P; Rodella, L; Somenza, E; Sorio, C; Tomba, F | 1 |
| Bakker, M; Beekman, JM; Berkers, G; Blaazer, AR; Brunsveld, JE; de Poel, E; de Winter de Groot, KM; Dompeling, E; Hagemeijer, MC; Heida-Michel, S; Ithakisiou, GN; Jans, SR; Koppelman, GH; Kruisselbrink, E; Muijlwijk-Koezen, JE; Oppelaar, H; Roukema, J; Spelier, S; Suen, SWF; van der Ent, CK; van der Meer, R; van Mourik, P; van Panhuis, H; Vonk, AM; Weersink, EJM | 1 |
| Amato, F; Castaldo, G; Hristodor, AM; Kleinfelder, K; Laudanna, C; Melotti, P; Sorio, C; Villella, VR | 1 |
| Beekman, JM; Berkers, G; Clevers, H; de Winter-de Groot, KM; Dekkers, JF; Elias, SG; Geerdink, M; Heijerman, HGM; Kruisselbrink, E; Marck-van der Wilt, REP; Michel, S; van der Ent, CK; van der Meer, R; Vleggaar, FP; Vonk, A; Vries, R | 1 |
| Gioino, P; Grishchenko, N; Hassan, N; Ianowski, JP; Jagadeeshan, S; Luan, X; Machen, TE; Shipley, AM; Tam, JS | 1 |
| Becq, F; Cantereau, A; Froux, L; Llinares, J | 1 |
| Beekman, JM; Graeber, SY; Hirtz, S; Kruisselbrink, E; Mall, MA; van der Ent, CK; van Mourik, P; Vonk, AM | 1 |
| Amaral, MD; Beekman, JM; Boj, SF; de Boeck, K; Dekkers, JF; Houwen, RHJ; Kruisselbrink, E; Mullenders, J; Ramalho, AS; Silva, IAL; Statia, M; Suen, SWF; van der Ent, CK; van Mourik, P; Vleggaar, FP; Vonk, AM; Vries, R | 1 |
| Aban, I; Boyles, SE; Chaudhry, IG; Chua, M; Gentzsch, M; Guimbellot, JS; Jaspers, I; Leach, JM; Quinney, NL | 1 |
| Boyles, SE; Cholon, DM; Gentzsch, M; Martino, MEB; Quinney, NL; Ribeiro, CMP | 1 |
| Harvey, BJ; Katzenellenbogen, JA; Kim, SH; Saint-Criq, V | 1 |
| Hwang, PH; Jeong, JH; Joo, NS; Wine, JJ | 1 |
| Edlund, A; Eliasson, L; Esguerra, JL; Flodström-Tullberg, M; Wendt, A | 1 |
| Flood, RD; Kozel, BJ; Luckie, DB; Malhotra, V; Massey, MK; Shah, AA; Van Alst, AJ | 1 |
| Kim, AH; Koval, M; McCarty, NA; Molina, SA; Moriarty, HK; Stauffer, B | 1 |
| Baekelandt, V; Beekman, JM; Bijvelds, MJ; Carlon, MS; da Cunha, MF; De Boeck, K; de Jonge, HR; Debyser, Z; Dekkers, JF; Edelman, A; Ferrante, M; Gijsbers, R; Hollenhorst, MI; Janssens, HM; Ramalho, AS; Sermet-Gaudelus, I; Van den Haute, C; Vidović, D | 1 |
| Birket, SE; Chu, KK; Fernandez, CM; Grizzle, WE; Hanes, J; Houser, GH; Lin, V; Liu, L; Mazur, M; Rowe, SM; Shastry, S; Sloane, PA; Solomon, GM; Sorscher, EJ; Tearney, GJ | 1 |
| Edlund, A; Eliasson, L; Flodström-Tullberg, M; Lindqvist, A; Pedersen, MG; Wierup, N | 1 |
| Beekman, JM; Boj, SF; Clevers, H; Statia, M; Su, J; Vonk, AM; Vries, RR | 1 |
| Al-Nakkash, L; Kurth, MJ; Nantz, MH; Springsteel, MF | 1 |
| Balossier, G; Coraux, C; Delavoie, F; Michel, J; Milliot, M; Molinari, M; Zahm, JM | 1 |
| Boucher, RC; Grubb, BR; Ostrowski, LE; Rogers, TD | 1 |
| Finkbeiner, WE; Mills, AD; Namkung, W; Padmawar, P; Song, Y; Verkman, AS | 1 |
| Choi, JY; Joo, NS; Khansaheb, M; Krouse, ME; Robbins, RC; Weill, D; Wine, JJ | 1 |
| Alper, SL; Bajmoczi, M; Gadjeva, M; Golan, DE; Pier, GB | 1 |
| Calvo, JJ; Case, RM; Colledge, WH; Fernández-Salazar, MP; García, M; Hernández-Lorenzo, MP; Pascua, P; San Román, JI; Steward, MC | 1 |
| Dragomir, A; Johannesson, M; Lazorova, L; Nilsson, HE; Roomans, GM | 1 |
| Bossard, F; Gauthier, C; Lavazais-Blancou, E; Robay, A; Rozec, B; Sagan, C; Silantieff, E | 1 |
| Clancy, JP; Collawn, J; Fan, L; Fortenberry, J; Fulton, J; Jurkevante, A; Li, Y; Mazur, M; Pyle, LC; Rowe, SM; Sloane, PA; Sorscher, EJ; Varga, K; Woodworth, B | 1 |
| Bompadre, SG; Hwang, TC; Li, M; Miki, H; Zhou, Z | 1 |
| Ackerley, CA; Bear, CE; Huan, LJ; Kim Chiaw, P; Lamhonwah, AM; Tein, I | 1 |
| Ballmann, M; Brandes, G; Derichs, N; Junge, S; Siebert, B; Stanke, F; Tamm, S; Tümmler, B; van Barneveld, A | 1 |
| Cho, HJ; Joo, NS; Khansaheb, M; Wine, JJ | 1 |
| Derichs, N; Finkbeiner, WE; Jin, BJ; Song, Y; Verkman, AS | 1 |
| Bolger, GB; Clancy, JP; Ehrhardt, A; Fan, L; Li, Y; Mitchell, LH; Naren, AP; Pyle, LC; Ren, A; Rowe, SM; Sorscher, EJ | 1 |
| Cho, HJ; Joo, NS; Wine, JJ | 1 |
| Fong, P; Ganta, S; Li, Y | 1 |
| Becq, F; Gimenez-Maitre, A; Guillaume, C; Jacquot, J; Laurent-Maquin, D; Le Henaff, C; Norez, C; Ravoninjatovo, B | 1 |
| Blouquit-Laye, S; Braun, C; Chinet, T; Dannhoffer, L; Dinh-Xuan, AT; Sage, E | 1 |
| Bridges, RJ | 1 |
| Atherton-Watson, H; Canning, P; Danahay, HL; de Courcey, F; Elborn, JS; Ennis, M; Williams, MT; Zholos, AV | 1 |
| Amaral, MD; Bertuzzo, CS; Bonadia, LC; Cardoso, SR; Felício, V; Kmit, A; Kunzelmann, K; Marson, FA; Ramalho, AS; Ribeiro, AF; Ribeiro, JD; Ribeiro, MA; Servidoni, MF; Sousa, L; Sousa, M; Uliyakina, I; Vinagre, AM | 1 |
| Galietta, LJ; Ma, T; Pedemonte, N; Verkman, AS; Vetrivel, L; Yang, H; Zegarra-Moran, O | 1 |
| Bebök, Z; Bedwell, DM; Colledge, WH; Dey, CR; Du, M; Evans, MJ; Jones, JR; Keeling, KM; Lanier, J; Lindsey, JR; Sorscher, EJ; Tousson, A; Whitsett, JA | 1 |
| Irokawa, T; Joo, NS; Robbins, RC; Whyte, RI; Wine, JJ; Wu, JV | 1 |
| Boucher, RC; Coakley, RD; Paradiso, AM | 1 |
| Clancy, JP; Cobb, BR; Fan, L; Kovacs, TE; Sorscher, EJ | 1 |
| Du, K; Folli, C; Galietta, LJ; Gopinath, VS; Guy, RK; Lukacs, GL; Ma, T; Pedemonte, N; Shelat, AA; Taddei, A; Verkman, AS; Yang, H | 1 |
| Ajonuma, LC; Chan, HC; Chan, LN; Chung, YW; Ho, LS; Lam, SY; Liu, Y; Lo, PS; Shi, QX; Tsang, LL; Wang, XF; Yu, MK; Yuan, YY; Zhao, WC; Zhou, CX | 1 |
| Cho, WK; Siegrist, VJ; Zinzow, W | 1 |
| Haggie, PM; Song, Y; Thiagarajah, JR; Verkman, AS | 1 |
| Galietta, LJ; Pedemonte, N; Qian, L; Salinas, D; Sonawane, ND; Song, Y; Verkman, AS | 1 |
| Barrett, KE; Childs, D; Chow, JY; Dong, H; Hogan, DL; Isenberg, JI; Pratha, VS; Sellers, ZM; Smith, AJ | 1 |
| Caci, E; Du, K; Galietta, LJ; Lukacs, GL; Pedemonte, N; Verkman, AS; Zegarra-Moran, O | 1 |
| Irokawa, T; Joo, NS; Robbins, RC; Wine, JJ | 1 |
| Colledge, WH; Riederer, B; Seidler, U; Soleimani, M; Tuo, B; Wang, Z | 1 |
| Kim, US; Nguyen, TD; Perrine, SP | 1 |
| Ballmann, M; Naim, HY; Stanke, F; Tümmler, B; van Barneveld, A | 1 |
| Blouquit, S; Boucher, R; Chinet, T; Dannhoffer, L; Fermanian, C; Naline, E; Regnier, A | 1 |
| Kerem, E | 1 |
| Cope, G; Cuthbert, AW; MacVinish, LJ; Ropenga, A | 1 |
| Burns, KA; Grubb, BR; Kulaga, HM; Ostrowski, LE; Reed, RR; Rogers, TD; Wonsetler, RL | 1 |
| De Jonge, H; Leal, T; Lebacq, J; Lebecque, P; Lecourt, H; Lubamba, B; Wallemacq, P | 1 |
| Bruscia, E; Caputo, C; Egan, ME; Ferreira, EC; Krause, DS; Price, JE; Weiner, SA | 1 |
| Bot, A; Breves, G; Burmester, M; Charizopoulou, N; de Jonge, HR; Dorsch, M; Hedrich, HJ; Jansen, S; Leonhard-Marek, S; Stanke, F; Tóth, B; Tümmler, B; Wedekind, D; Wilke, M | 1 |
| Dho, S; Fedorko, L; Foskett, JK; Marunaka, Y; O'Brodovich, H; Wang, X | 1 |
| Fischer, H; Illek, B; Machen, TE; Poulsen, JH | 1 |
| Reenstra, WW; Santos, GF | 1 |
| Dey, CR; DuVall, MD; Frizzell, RA; Wert, SE; Whitsett, JA; Zhou, L | 1 |
| Fischer, H; Machen, TE | 1 |
| Bear, CE; Krolczyk, AJ; Lai, PF; Schimmer, BP | 1 |
| Banks, TC; Crystal, RG; Danel, C; Rosenfeld, MA; Rosenfeld, SJ | 1 |
| Boucher, RC; Gabriel, SE; Leung, AY; Wong, PY; Yankaskas, JR | 1 |
| Grubb, BR | 2 |
| Oz, MC; Sorota, S | 1 |
| Baró, I; Escande, D; Hongre, AS; Roch, B | 1 |
| Hume, JR; Levesque, PC | 1 |
| Frömter, E; Schröder, UH | 2 |
| Besmond, C; Goossens, M; Vega, MA | 1 |
| Augustinas, O; Becq, F; Chang, XB; Hanrahan, JW; Jensen, TJ; Mathews, CJ; Riordan, JR; Tabcharani, JA | 1 |
| Basavappa, S; Cohn, JA; Fitz, JG; McGill, J; Melhus, O | 1 |
| Marchase, RB; Prince, LS; Tousson, A | 1 |
| Colledge, WH; Cuthbert, AW; Evans, MJ; Gill, DR; Higgins, CF; Hyde, SC; MacVinish, LJ; Ratcliff, R; Trezise, AE | 1 |
| Breuer, WV; Cabantchik, ZI; Greger, R; Slotki, IN | 1 |
| Boucher, R; Grubb, B; Knowles, M; Lazarowski, E | 1 |
| Kersting, D; Kersting, U; Spring, KR | 1 |
| Boucher, RC; Clarke, LL; Gabriel, SE; Stutts, MJ | 1 |
| Boucher, RC; Gabriel, SE; Gatzy, JT; O'Connell, TL; Olsen, JC; Price, EM; Stutts, MJ | 1 |
| Benos, DJ; Cunningham, SA; Frizzell, RA; Morris, AP | 2 |
| Colledge, WH; Cuthbert, AW; Evans, MJ; Halstead, J; Ratcliff, R | 1 |
| Horie, M; Okada, Y; Tominaga, M | 1 |
| Chapman, T; Collier, ML; Geary, Y; Hart, P; Horowitz, B; Hume, JR; Levesque, PC; Warth, J | 1 |
| Colledge, WH; Cuthbert, AW; Evans, MJ; Hickman, ME; MacVinish, LJ; Ratcliff, R | 1 |
| Boucher, RC; Grubb, BR; Paradiso, AM | 1 |
| Baró, I; Berthon, B; Escande, D; Hongre, AS | 1 |
| Alton, EW; Coutelle, C; Frost, A; Hart, SL; Huang, L; Mayall, E; Munkonge, FM; Stern, M; Vasilliou, M; Williamson, R | 1 |
| Fang, RH; Krouse, ME; Wine, JJ; Xia, Y | 1 |
| Biwersi, J; Seksek, O; Verkman, AS | 1 |
| Alton, EW; Caplen, NJ; Geddes, DM; Huang, L; Munkonge, FM; Sorgi, F; Stern, M | 1 |
| Boucher, RC; Leung, AY; Wong, PY; Yankaskas, JR | 1 |
| Figarella, C; Merten, M; Montserrat, C | 1 |
| Alton, EW; Delaney, SJ; Dorin, JR; Farini, E; Farley, R; Porteous, DJ; Smith, SN; Wainwright, BJ; Webb, S | 1 |
| Galietta, LJ; Romano, L; Rossi, GA; Sacco, O; Zegarra-Moran, O | 1 |
| Bear, CE; Durie, P; Gyömörey, K; Nguyen, V; Plyte, S; Rozmahel, R; Tsui, LC; Wilschanski, M | 1 |
| Birchall, N; Colledge, WH; Cuthbert, AW; Episkopou, V; Evans, MJ; Huxley, C; Kasschau, KD; MacVinish, LJ; Manson, AL; Trezise, AE; Vassaux, G | 1 |
| Egan, ME; Rubenstein, RC; Zeitlin, PL | 1 |
| Cotton, CU; Drumm, ML; Kelley, TJ | 1 |
| Crystal, RG; Singh, RN; Suzuki, M | 1 |
| Alton, EW; Delaney, SJ; Dorin, JR; Farley, R; Geddes, DM; Porteous, DJ; Smith, SN; Wainwright, BJ | 1 |
| Cuthbert, AW; Huxley, C | 1 |
| Braganza, JM; Jaran, AS; Quesnel, LB | 1 |
| Cuthbert, AW; Durrington, HJ; Hickman, ME; MacVinish, LJ; Mufti, DA | 1 |
| Colledge, WH; Curtis, CM; Cuthbert, AW; Evans, MJ; Hickman, ME; Higgins, CF; MacVinish, LJ; Martin, LC | 1 |
| Brahimi-Horn, MC; Capeau, J; Mailleau, C | 1 |
| O'Farrell, AM; O'Reilly, CM; Ryan, MP | 1 |
| Bragin, A; Devidas, S; Engelhardt, JF; Foskett, JK; Guggino, WB; Jiang, Q; Mak, D; Schwiebert, EM; Skach, WR; Zhang, Y | 1 |
| Cuthbert, AW; Hickman, ME; MacVinish, LJ | 1 |
| Al-Nakkash, L; Hwang, TC | 1 |
| Beharry, S; Durie, PR; Kent, G; Tang, S | 1 |
| Shumaker, H; Soleimani, M | 1 |
| Bridges, RJ; Devor, DC; Gerlach, AC; Gondor, M; Pilewski, JM; Singh, AK | 1 |
| Hurlock, G; Moss, RB; Robinson, C; Wine, JJ; Yee, K | 1 |
| Hu, S; Hwang, TC; Wang, F; Zeltwanger, S | 1 |
| Argent, BE; Gray, MA; O'Reilly, CM; Winpenny, JP | 1 |
| Clarke, LL; Gawenis, LR; Harline, MC; Turner, JT; Walker, NM; Weisman, GA | 1 |
| Brandis, M; Greger, R; Hübner, M; Kuehr, J; Kunzelmann, K; Mall, M; Seydewitz, HH; Wissner, A | 1 |
| Brandis, M; Greger, R; Kuehr, J; Kunzelmann, K; Mall, M; Schreiber, R; Seydewitz, HH; Wissner, A | 1 |
| Harvey, BJ; Urbach, V; Walsh, DE | 1 |
| Bebök, Z; Schwiebert, LM; Tousson, A; Venglarik, CJ | 1 |
| Cuthbert, AW | 1 |
| Cuppoletti, J; Kupert, EY; Malinowska, DH; Sherry, AM; Tewari, KP | 1 |
| Barbry, P; Fischer, H; Fukuda, N; Illek, B; Matthay, MA; Sartori, C | 1 |
| Krouse, ME; Wine, JJ | 1 |
| Andersson, C; Aslund, M; Dragomir, A; Hjelte, L; Roomans, GM | 1 |
| Brady, KG; Drumm, ML; Kelley, TJ | 1 |
| Duszyk, M | 1 |
| Bear, CE; Galley, K; Garami, E; Gyömörey, K; Rommens, JM | 1 |
| Cuthbert, AW; Keogh, J; MacVinish, LJ | 1 |
| Alton, EW; Hume, DA; Kunzelmann, K; McMorran, BJ; Oceandy, D; Schreiber, R; Smith, SN; Wainwright, BJ | 1 |
| Gruenert, DC; Haws, C; Krouse, ME; Wine, JJ; Xia, Y | 1 |
| Bell, CL; Quinton, PM; Reddy, MM | 1 |
| Smith, JJ; Welsh, MJ | 1 |
| Alton, EW; Geddes, DM; Smith, SN | 1 |
| Bell, CL; Quinton, PM | 1 |
| Benos, DJ; Cunningham, SA; Frizzell, RA; Worrell, RT | 1 |
| Root, KV; Schreiber, JH; Van Dyke, RW; Wilson, JM | 1 |
| Berta, G; Bradbury, NA; Bridges, RJ; Jilling, T; Kirk, KL; Sorscher, EJ | 1 |
| Breuer, W; Cabantchik, ZI; Kartner, N; Riordan, JR | 1 |
| Chang, XB; Grinstein, S; Kartner, N; Lukacs, GL; Riordan, JR; Rotstein, OD | 1 |
| Boucher, RC; Clarke, LL; Gabriel, SE; Grubb, BR; Koller, BH; Smithies, O | 1 |
| Bradbury, NA; Bridges, RJ | 1 |
| Brayden, DJ; Cuthbert, AW; Pickles, RJ | 1 |
| Pedersen, PS | 1 |
| Barker, PE; Cunningham, S; Frizzell, RA; Green, MW; Jilling, T; Kirk, KL | 1 |
| Dorkin, HL; Grubman, SA; Iannuzzi, MC; Jefferson, DM; Klinger, KW; Li, M; Marini, FC; Valentich, JD; Welsh, MJ | 1 |
| Barbry, P; Champigny, G; Chastre, E; Di Gioia, Y; Emami, S; Fanen, P; Gespach, C; Mornet, E; Simon-Bouy, B | 1 |
| Bertrand, F; Capeau, J; Cherqui, G; Garcia, I; Hermelin, B; Paul, A; Picard, J | 1 |
| Banks, TC; Bargon, J; Chu, CS; Dalemans, W; Guggino, WB; Nakamura, H; Pavirani, A; Trapnell, BC; Yoshimura, K; Zeitlin, PL | 1 |
| Boucher, RC; Jetten, AM; Stutts, MJ; Willumsen, NJ; Yankaskas, JR | 1 |
| Chevallier, I; Krause, KH; Lew, DP; Suter, S | 1 |
| Martinez, AM; Martinez, JR | 1 |
3 review(s) available for colforsin and Cystic Fibrosis
| Article | Year |
|---|---|
Mechanisms of bicarbonate secretion: lessons from the airways.
Topics: Benzimidazoles; Bicarbonates; Bronchi; Calcium; Calcium Channel Agonists; Calcium Channels; Cell Line; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Impedance; Humans; Intermediate-Conductance Calcium-Activated Potassium Channels; Membrane Potentials; Microelectrodes; Serous Membrane | 2012 |
Mutation specific therapy in CF.
Topics: Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA; Enzyme Inhibitors; Genistein; Gentamicins; Humans; Mutation; Treatment Outcome | 2006 |
Function and dysfunction of the CFTR chloride channel.
Topics: Adenosine Triphosphate; Animals; Chloride Channels; Colforsin; Cystic Fibrosis; Hydrolysis; Phosphorylation | 1995 |
167 other study(ies) available for colforsin and Cystic Fibrosis
| Article | Year |
|---|---|
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis.
Topics: Animals; Bronchi; Cell Membrane; Cricetinae; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Gene Silencing; Histone Deacetylases; Humans; Hydroxamic Acids; Mutation; Protein Denaturation; Protein Folding; RNA, Small Interfering; Vorinostat | 2010 |
Combined agonists act synergistically to increase mucociliary clearance in a cystic fibrosis airway model.
Topics: Animals; Carbachol; Colforsin; Cystic Fibrosis; Disease Models, Animal; Drug Synergism; Drug Therapy, Combination; Ferrets; Isoproterenol; Mucociliary Clearance; Muscle Contraction; Muscle, Smooth; Swine | 2021 |
cAMP triggers Na
Topics: 1-Methyl-3-isobutylxanthine; Amiloride; Animals; Animals, Genetically Modified; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Epithelial Sodium Channel Blockers; Epithelial Sodium Channels; Epithelium; Ion Transport; Male; Sodium; Swine | 2021 |
Forskolin-induced organoid swelling is associated with long-term cystic fibrosis disease progression.
Topics: Biomarkers; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Exocrine Pancreatic Insufficiency; Humans; Mutation; Organoids | 2022 |
Forskolin induced swelling (FIS) assay in intestinal organoids to guide eligibility for compassionate use treatment in a CF patient with a rare genotype.
Topics: Aminophenols; Benzodioxoles; Colforsin; Compassionate Use Trials; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genotype; Humans; Middle Aged; Mutation; Organoids; Rectum | 2022 |
Evaluation of the Complex p.[Leu467Phe;Phe508del] CFTR Allele in the Intestinal Organoids Model: Implications for Therapy.
Topics: Alleles; Benzodioxoles; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Ligases; Mutation; Organoids | 2022 |
CFTR Modulators Rescue the Activity of CFTR in Colonoids Expressing the Complex Allele p.[R74W;V201M;D1270N]/dele22_24.
Topics: Alleles; Benzodioxoles; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Middle Aged; Mutation | 2023 |
FDA-approved drug screening in patient-derived organoids demonstrates potential of drug repurposing for rare cystic fibrosis genotypes.
Topics: Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Evaluation, Preclinical; Drug Repositioning; Genotype; Humans; Mutation; Organoids; Phosphodiesterase 4 Inhibitors | 2023 |
Theratyping of the Rare CFTR Genotype A559T in Rectal Organoids and Nasal Cells Reveals a Relevant Response to Elexacaftor (VX-445) and Tezacaftor (VX-661) Combination.
Topics: Benzodioxoles; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Humans; Mutation; Organoids | 2023 |
Forskolin-induced swelling of intestinal organoids correlates with disease severity in adults with cystic fibrosis and homozygous F508del mutations.
Topics: Adjuvants, Immunologic; Adult; Biopsy; Colforsin; Correlation of Data; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Mutation; Nutritional Status; Organoids; Rectum; Respiratory Function Tests; Severity of Illness Index | 2020 |
Airway submucosal glands from cystic fibrosis swine suffer from abnormal ion transport across the serous acini, collecting duct, and ciliated duct.
Topics: Acinar Cells; Animals; Carbachol; Cations, Monovalent; Chlorides; Cilia; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Electrochemical Techniques; Electrodes; Gene Deletion; Gene Expression; Humans; Ion Transport; Lung; Potassium; Sodium; Swine | 2020 |
Quantitative phase imaging to study transmembrane water fluxes regulated by CFTR and AQP3 in living human airway epithelial CFBE cells and CHO cells.
Topics: Aminophenols; Animals; Aquaporin 3; Biological Transport, Active; Biophysical Phenomena; Bronchi; Cell Line; Chloride Channel Agonists; CHO Cells; Colforsin; Cricetulus; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Microscopy, Interference; Mutant Proteins; Osmosis; Quinolones; Respiratory Mucosa; RNA, Messenger; Water | 2020 |
Comparison of Organoid Swelling and
Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Edema; Female; Forced Expiratory Volume; Homozygote; Humans; In Vitro Techniques; Male; Membrane Potentials; Middle Aged; Nasal Mucosa; Organoids; Precision Medicine; Prospective Studies; Quinolones; Rectum; Sweat; Treatment Outcome; Vital Capacity; Young Adult | 2020 |
Protocol for Application, Standardization and Validation of the Forskolin-Induced Swelling Assay in Cystic Fibrosis Human Colon Organoids.
Topics: Biological Assay; Cells, Cultured; Colforsin; Colon; Cystic Fibrosis; Edema; Humans; Organoids; Reproducibility of Results | 2020 |
Nasospheroids permit measurements of CFTR-dependent fluid transport.
Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biological Transport; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Mutation; Nasal Mucosa; Particle Size; Precision Medicine; Quinolones; Spheroids, Cellular | 2017 |
The cystic fibrosis airway milieu enhances rescue of F508del in a pre-clinical model.
Topics: Aminophenols; Aminopyridines; Benzodioxoles; Bronchi; Cell Line; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Inflammation; Mutation; Quinolones; Respiratory System | 2018 |
Non-genomic estrogen regulation of ion transport and airway surface liquid dynamics in cystic fibrosis bronchial epithelium.
Topics: Amiloride; Bronchi; Bumetanide; Cell Line; Colforsin; Cyclic AMP; Cystic Fibrosis; Epithelial Cells; Epithelial Sodium Channels; Estradiol; Female; Gene Expression Regulation; Humans; Ion Transport; Male; Ouabain; Protein Kinase C-delta; Respiratory Mucosa; Signal Transduction; Sodium; Sodium-Potassium-Exchanging ATPase; Water | 2013 |
Mucociliary clearance and submucosal gland secretion in the ex vivo ferret trachea.
Topics: Analgesics, Non-Narcotic; Angiogenesis Inhibitors; Animals; Bronchodilator Agents; Bumetanide; Calcium; Carbachol; Carbonates; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exocrine Glands; Female; Ferrets; Ion Transport; Isoproterenol; Mucociliary Clearance; Mucus; Nitrobenzoates; Sodium Potassium Chloride Symporter Inhibitors; Trachea | 2014 |
CFTR and Anoctamin 1 (ANO1) contribute to cAMP amplified exocytosis and insulin secretion in human and murine pancreatic beta-cells.
Topics: Animals; Anoctamin-1; Calcium; Calcium Channels; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exocytosis; Glucagon-Like Peptide 1; Glycine; Humans; Hydrazines; Insulin-Secreting Cells; Insulins; Ion Channels; Mice; Neoplasm Proteins; Patch-Clamp Techniques | 2014 |
Chemical rescue of ΔF508-CFTR in C127 epithelial cells reverses aberrant extracellular pH acidification to wild-type alkalization as monitored by microphysiometry.
Topics: Animals; Bicarbonates; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Hydrogen-Ion Concentration; Mice; Mice, Inbred CFTR; NIH 3T3 Cells | 2014 |
Junctional abnormalities in human airway epithelial cells expressing F508del CFTR.
Topics: Adult; Calcium Signaling; Cell Line; Colforsin; Connexin 26; Connexin 43; Connexins; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Gap Junctions; Humans; Male; Phenylbutyrates; Protein Transport; Respiratory Mucosa; Tight Junctions | 2015 |
rAAV-CFTRΔR Rescues the Cystic Fibrosis Phenotype in Human Intestinal Organoids and Cystic Fibrosis Mice.
Topics: Animals; Body Fluids; Chloride Channels; Chlorides; Colforsin; Cystic Fibrosis; Dependovirus; Disease Models, Animal; Gene Transfer Techniques; Genetic Therapy; Genetic Vectors; Genotype; HeLa Cells; Humans; Intestines; Mice; Organoids; Transduction, Genetic | 2016 |
Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy.
Topics: Amiloride; Aminophenols; Animals; Cells, Cultured; Chloride Channel Agonists; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Evaluation, Preclinical; Drug Therapy, Combination; Humans; Membrane Potentials; Mice; Mutation, Missense; NIH 3T3 Cells; Quinolones | 2016 |
CFTR is involved in the regulation of glucagon secretion in human and rodent alpha cells.
Topics: Animals; Cells, Cultured; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Glucagon; Glucagon-Secreting Cells; Glucose; Humans; Male; Membrane Potentials; Mice; Middle Aged; Mutation; Somatostatin-Secreting Cells | 2017 |
Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients.
Topics: Biological Assay; Cells, Cultured; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Intestinal Mucosa; Ion Transport; Mutation; Organoids; Vasodilator Agents | 2017 |
Activation of CFTR by UCCF-029 and genistein.
Topics: Adenosine Triphosphate; Animals; Benzoflavones; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Drug Design; Genistein; Hydrolysis; Mice; Models, Chemical; NIH 3T3 Cells; Protein Kinase Inhibitors; Transfection | 2008 |
Salmeterol restores secretory functions in cystic fibrosis airway submucosal gland serous cells.
Topics: Albuterol; Cell Line; Cell Polarity; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrophysiological Phenomena; Epithelial Cells; Exocytosis; Humans; Intracellular Space; Ions; Muramidase; Salmeterol Xinafoate; Secretory Vesicles; Serous Membrane; Trachea | 2009 |
Ion transport across CF and normal murine olfactory and ciliated epithelium.
Topics: Age Factors; Amiloride; Animals; Chlorides; Cilia; Colforsin; Cystic Fibrosis; Disease Models, Animal; Humans; Ion Transport; Male; Membrane Potentials; Mice; Mice, Inbred BALB C; Mice, Inbred C57BL; Mice, Inbred CFTR; Nasal Cavity; Nasopharynx; Olfactory Mucosa; Respiratory Mucosa; Sodium; Tissue Culture Techniques | 2009 |
In situ measurement of airway surface liquid [K+] using a ratioable K+-sensitive fluorescent dye.
Topics: Adenosine Triphosphate; Amiloride; Animals; Bronchi; Cells, Cultured; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Primers; Electrophysiology; Epithelial Cells; Fluorescent Dyes; Humans; Mice; Microscopy, Fluorescence; Potassium; Potassium Channels; Reverse Transcriptase Polymerase Chain Reaction; RNA; RNA, Messenger; Trachea | 2009 |
Substance P stimulates human airway submucosal gland secretion mainly via a CFTR-dependent process.
Topics: Age Factors; Animals; Calcium Signaling; Capsicum; Carbachol; Chelating Agents; Clotrimazole; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Drug Synergism; Egtazic Acid; Exocrine Glands; Female; Humans; In Vitro Techniques; Male; Mucus; Plant Oils; Substance P; Sus scrofa; Trachea; Vasoactive Intestinal Peptide | 2009 |
Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas aeruginosa.
Topics: Aminoglycosides; Animals; Caveolin 1; Cell Line; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Endocytosis; Epithelial Cells; Humans; Immunity, Innate; Membrane Microdomains; Pseudomonas aeruginosa; Pseudomonas Infections; Recombinant Fusion Proteins; Respiratory Tract Infections; RNA, Small Interfering | 2009 |
Ducts isolated from the pancreas of CFTR-null mice secrete fluid.
Topics: Animals; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Mice; Mice, Inbred CFTR; Pancreatic Ducts; Secretin | 2009 |
CFTR and tight junctions in cultured bronchial epithelial cells.
Topics: 1-Methyl-3-isobutylxanthine; Actins; Bronchi; Cell Line; Cell Membrane Permeability; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Cytoskeleton; Electric Conductivity; Humans; Lanthanum; Mannitol; Respiratory Mucosa; Tight Junctions; Time Factors; Tubulin | 2010 |
β1, β2, and β3 adrenoceptors and Na+/H+ exchanger regulatory factor 1 expression in human bronchi and their modifications in cystic fibrosis.
Topics: Adrenergic beta-Agonists; Adult; Aged; Albuterol; Benzoates; Bronchi; Case-Control Studies; Cell Line; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dobutamine; Female; Gene Expression Regulation; Humans; Male; Middle Aged; Phosphoproteins; Prazosin; Receptors, Adrenergic, beta-1; Receptors, Adrenergic, beta-2; Receptors, Adrenergic, beta-3; RNA, Messenger; Sodium-Hydrogen Exchangers; Thiazolidines; Young Adult | 2011 |
DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers.
Topics: Animals; Cells, Cultured; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Genistein; Humans; Ion Transport; Phosphodiesterase Inhibitors; Phosphoric Monoester Hydrolases; Rats; Rats, Inbred F344; Temperature | 2010 |
Potentiation of disease-associated cystic fibrosis transmembrane conductance regulator mutants by hydrolyzable ATP analogs.
Topics: Adenosine Triphosphate; Amino Acid Substitution; Animals; Cell Line, Tumor; CHO Cells; Colforsin; Cricetinae; Cricetulus; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Synergism; Genistein; Humans; Ion Channel Gating; Membrane Potentials; Mutation; Patch-Clamp Techniques; Transfection | 2010 |
Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise.
Topics: Animals; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exercise; Female; Gene Expression Regulation; Humans; Magnetic Resonance Spectroscopy; Mice; Mice, Inbred C57BL; Mice, Transgenic; Microscopy, Immunoelectron; Muscle, Skeletal; Mutation; Phosphodiesterase Inhibitors; Sarcolemma; Subcellular Fractions | 2010 |
Functional analysis of F508del CFTR in native human colon.
Topics: Adolescent; Adult; Child; Chlorides; Colforsin; Colon; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Glycosylation; Homozygote; Humans; Immunoblotting; Intestinal Mucosa; Ion Transport; Lung; Mutant Proteins; Mutation; Respiratory Mucosa; Young Adult | 2010 |
Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs.
Topics: Animals; Animals, Newborn; Body Fluids; Carbachol; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Exocrine Glands; Respiratory System; Substance P; Sus scrofa; Trachea | 2010 |
Hyperviscous airway periciliary and mucous liquid layers in cystic fibrosis measured by confocal fluorescence photobleaching.
Topics: Amiloride; Bronchi; Cells, Cultured; Cilia; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dextrans; Epithelial Cells; Epithelial Sodium Channel Blockers; Epithelial Sodium Channels; Fluorescein-5-isothiocyanate; Fluorescence Recovery After Photobleaching; Humans; Microscopy, Confocal; Mucus; Respiratory Mucosa; Viscosity | 2011 |
Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators.
Topics: Aminophenols; Animals; Blotting, Western; Cell Line; Chlorocebus aethiops; Colforsin; Cresols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diffusion Chambers, Culture; Dimerization; Enzyme-Linked Immunosorbent Assay; Gene Expression; High-Throughput Screening Assays; Humans; Ion Channel Gating; Ion Transport; Lentivirus; Membrane Transport Modulators; Mice; Mutation; Phosphorylation; Protein Structure, Tertiary; Pyrazoles; Quinolones; Retroviridae; Transduction, Genetic | 2011 |
Defective fluid secretion from submucosal glands of nasal turbinates from CFTR-/- and CFTR (ΔF508/ΔF508) pigs.
Topics: Aging; Animals; Animals, Newborn; Body Fluids; Carbachol; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Synergism; Exocrine Glands; Mutation; Organ Size; Substance P; Sus scrofa; Trachea; Turbinates | 2011 |
Endogenous surface expression of ΔF508-CFTR mediates cAMP-stimulated Cl(-) current in CFTR(ΔF508/ΔF508) pig thyroid epithelial cells.
Topics: Amiloride; Animals; Animals, Genetically Modified; Anions; Cells, Cultured; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Gene Knockout Techniques; Ion Transport; Membrane Potentials; Membrane Proteins; Mutation; Protein Folding; Protein Transport; Proteolysis; Sus scrofa; Swine; Temperature; Thyroid Gland | 2012 |
Deficit of osteoprotegerin release by osteoblasts from a patient with cystic fibrosis.
Topics: Adenylyl Cyclases; Adult; Cells, Cultured; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genistein; Humans; Male; Osteoblasts; Osteoprotegerin; Tumor Necrosis Factor-alpha | 2012 |
Effect of nitric oxide on epithelial ion transports in noncystic fibrosis and cystic fibrosis human proximal and distal airways.
Topics: Adenosine Triphosphate; Adult; Aged; Amiloride; Bronchi; Chloride Channels; Colforsin; Cyclic GMP; Cyclic N-Oxides; Cystic Fibrosis; Epithelial Sodium Channel Blockers; Epithelial Sodium Channels; Free Radical Scavengers; Guanylate Cyclase; Humans; Imidazoles; Middle Aged; Nitric Oxide; Nitric Oxide Donors; Nitroprusside; Oxadiazoles; Purinones; Quinoxalines; Young Adult | 2012 |
Development of primary human nasal epithelial cell cultures for the study of cystic fibrosis pathophysiology.
Topics: Adult; Amiloride; Cells, Cultured; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Sodium Channel Blockers; Female; Humans; Interleukin-1beta; Interleukin-8; Lipopolysaccharides; Male; Nasal Lavage Fluid; Nasal Mucosa; Tumor Necrosis Factor-alpha; Uridine Triphosphate; Young Adult | 2012 |
Measurements of CFTR-mediated Cl- secretion in human rectal biopsies constitute a robust biomarker for Cystic Fibrosis diagnosis and prognosis.
Topics: 1-Methyl-3-isobutylxanthine; Biomarkers; Biopsy; Carbachol; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Humans; Ion Channel Gating; Prognosis; Rectum; Treatment Outcome | 2012 |
High-affinity activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening.
Topics: Animals; Bacterial Proteins; Cells, Cultured; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Evaluation, Preclinical; Epithelial Cells; Equipment Design; Genes, Reporter; Humans; Iodides; Kinetics; Luminescent Proteins; Rats; Rats, Inbred F344; Recombinant Fusion Proteins; Respiratory Mucosa; Structure-Activity Relationship; Thyroid Gland | 2002 |
Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.
Topics: Action Potentials; Animals; Anti-Bacterial Agents; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Gene Expression; Gentamicins; Heterozygote; Humans; Intestinal Mucosa; Mice; Mice, Knockout; Mice, Transgenic; Mutation; Promoter Regions, Genetic; RNA, Messenger; Suppression, Genetic; Tobramycin; Transgenes | 2002 |
Absent secretion to vasoactive intestinal peptide in cystic fibrosis airway glands.
Topics: Adult; Biopsy; Bronchi; Carbachol; Colforsin; Cystic Fibrosis; Female; Humans; Kinetics; Lung Transplantation; Male; Middle Aged; Respiratory Mucosa; Tissue Donors; Trachea; Vasoactive Intestinal Peptide | 2002 |
Polarized distribution of HCO3- transport in human normal and cystic fibrosis nasal epithelia.
Topics: 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; Adult; Amiloride; Barium; Bicarbonates; Biological Transport, Active; Cells, Cultured; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diffusion Chambers, Culture; Diuretics; Epithelial Cells; Female; Fluoresceins; Fluorometry; Humans; Hydrogen-Ion Concentration; Male; Nasal Mucosa; ortho-Aminobenzoates | 2003 |
Adenosine receptors and phosphodiesterase inhibitors stimulate Cl- secretion in Calu-3 cells.
Topics: Adenosine Deaminase; Anions; Cell Line; Chlorine; Cilostazol; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrophysiology; Epithelium; Glyburide; Humans; Hypoglycemic Agents; Milrinone; Mutation; Papaverine; Phosphodiesterase Inhibitors; Piperazines; Purines; Receptors, Purinergic P1; Rolipram; Sildenafil Citrate; Sulfones; Tetrazoles; Time Factors | 2003 |
Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating.
Topics: Animals; Biological Transport; Cell Line; Colforsin; Cricetinae; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Genistein; Humans; Iodides; Ion Channel Gating; Kinetics; Phenylalanine; Rats; Rats, Inbred F344; Recombinant Proteins; Respiratory Mucosa; Sequence Deletion; Thermodynamics; Thyroid Gland; Transfection | 2003 |
Involvement of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm.
Topics: Animals; Bicarbonates; Cells, Cultured; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Endometrium; Enzyme Inhibitors; Epithelial Cells; Female; Fertilization; Genistein; Humans; Male; Mice; Oocytes; Sperm Capacitation; Sperm-Ovum Interactions; Spermatozoa; Uterus | 2003 |
Impaired regulatory volume decrease in freshly isolated cholangiocytes from cystic fibrosis mice: implications for cystic fibrosis transmembrane conductance regulator effect on potassium conductance.
Topics: 1-Methyl-3-isobutylxanthine; Angiogenesis Inhibitors; Animals; Bile Ducts; Buffers; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; HEPES; Immunohistochemistry; Ionophores; Liver; Male; Mice; Mice, Inbred C57BL; Mice, Transgenic; Microscopy, Fluorescence; Microscopy, Video; Nitrobenzoates; Potassium; Sodium Chloride; Time Factors; Valinomycin | 2004 |
A small molecule CFTR inhibitor produces cystic fibrosis-like submucosal gland fluid secretions in normal airways.
Topics: Animals; Benzoates; Body Fluids; Bronchi; Cells, Cultured; Chlorides; Cholinergic Agents; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exocrine Glands; Humans; Hydrogen-Ion Concentration; Pilocarpine; Second Messenger Systems; Sodium; Swine; Thapsigargin; Thiazoles; Thiazolidines; Viscosity | 2004 |
Evidence against the rescue of defective DeltaF508-CFTR cellular processing by curcumin in cell culture and mouse models.
Topics: Administration, Oral; Amiloride; Animals; Anti-Inflammatory Agents, Non-Steroidal; Cell Cycle; Cell Line; Cells, Cultured; Chlorine; Chromatography, High Pressure Liquid; Colforsin; Curcumin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Homozygote; Humans; Mice; Mutation; Perfusion; Phenylbutyrates; Rats; Rats, Inbred F344; Temperature; Thyroid Gland; Time Factors; Trachea; Transfection | 2004 |
Heat-stable enterotoxin of Escherichia coli stimulates a non-CFTR-mediated duodenal bicarbonate secretory pathway.
Topics: Animals; Bacterial Toxins; Benzoquinones; Bicarbonates; Cell Membrane; Chloride-Bicarbonate Antiporters; Colforsin; Cyclic GMP; Cystic Fibrosis; Duodenum; Enterotoxins; Enzyme Inhibitors; Escherichia coli Proteins; Genistein; In Vitro Techniques; Lactams, Macrocyclic; Mice; Natriuretic Peptides; Peptides; Protein-Tyrosine Kinases; Quinones; Rifabutin | 2005 |
Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.
Topics: Animals; Cells, Cultured; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Design; Enzyme Inhibitors; Epithelial Cells; Genistein; Humans; Iodides; Luminescent Proteins; Molecular Structure; Mutation; Pyrimidinones; Respiratory Mucosa; Thiazoles | 2005 |
Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands.
Topics: Amiloride; Animals; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Primers; Exocrine Glands; Humans; Ion Transport; Lung; Polymerase Chain Reaction; Respiratory Mucosa; Swine; Time Factors; Tissue Distribution; Trachea | 2006 |
Involvement of the anion exchanger SLC26A6 in prostaglandin E2- but not forskolin-stimulated duodenal HCO3- secretion.
Topics: Animals; Antiporters; Bicarbonates; Carbachol; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dinoprostone; Disease Models, Animal; Duodenum; Humans; Intestinal Mucosa; Mice; Mice, Inbred Strains; Mice, Knockout; Sulfate Transporters | 2006 |
Novel short chain fatty acids restore chloride secretion in cystic fibrosis.
Topics: Cell Line; Chlorides; Colforsin; Cystic Fibrosis; Fatty Acids, Volatile; Humans; Iodine; Molecular Structure | 2006 |
Ex vivo biochemical analysis of CFTR in human rectal biopsies.
Topics: Adolescent; Adult; Biopsy; Carbachol; Child; Child, Preschool; Colforsin; Cysteine; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrophysiology; Histamine; Humans; Immunoprecipitation; In Vitro Techniques; Intestinal Mucosa; Isotope Labeling; Middle Aged; Rectum; Sulfur Radioisotopes | 2006 |
Ion and fluid transport properties of small airways in cystic fibrosis.
Topics: Body Water; Bronchi; Cells, Cultured; Chlorides; Colforsin; Cystic Fibrosis; Electric Impedance; Epithelial Cells; Humans; Ion Transport; Microscopy, Confocal; Middle Aged; Reference Values | 2006 |
Chloride transporting capability of Calu-3 epithelia following persistent knockdown of the cystic fibrosis transmembrane conductance regulator, CFTR.
Topics: Adenylyl Cyclases; Alprostadil; Carbachol; Cell Line; Chloride Channels; Chlorides; CLC-2 Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Enzyme Activation; Epithelial Cells; Fatty Acids; Genetic Vectors; Humans; Lubiprostone; Lung; Membrane Potentials; Muscarinic Agonists; Retroviridae; RNA Interference; RNA, Small Interfering; Time Factors | 2007 |
Olfactory epithelia exhibit progressive functional and morphological defects in CF mice.
Topics: Acetophenones; Adenylyl Cyclases; Aging; Aldehydes; Amiloride; Animals; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Dose-Response Relationship, Drug; Enzyme Activators; Mice; Mice, Inbred CFTR; Microscopy, Electron, Scanning; Odorants; Olfactory Mucosa; Olfactory Receptor Neurons; Pentanols; Receptors, Odorant; RNA, Messenger; Smell; Sodium; Sodium Channel Blockers; Time Factors; Tissue Culture Techniques | 2007 |
Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis.
Topics: Animals; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Imidazoles; Injections, Intraperitoneal; Ion Transport; Membrane Potentials; Mice; Mice, Inbred CFTR; Nasal Mucosa; Phosphodiesterase 5 Inhibitors; Phosphodiesterase Inhibitors; Piperazines; Protein Modification, Translational; Purines; Sildenafil Citrate; Sulfones; Triazines; Vardenafil Dihydrochloride | 2008 |
Rectal potential difference and the functional expression of CFTR in the gastrointestinal epithelia in cystic fibrosis mouse models.
Topics: Animals; Benzoates; Colforsin; Colon; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Genotype; Intestinal Mucosa; Membrane Potentials; Mice; Mice, Inbred CFTR; Mice, Mutant Strains; Phenotype; Rectum; Reproducibility of Results; Thiazolidines | 2008 |
Very mild disease phenotype of congenic CftrTgH(neoim)Hgu cystic fibrosis mice.
Topics: Analysis of Variance; Animals; Body Weight; Carbachol; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Female; Fertility; Genetic Linkage; Genotype; Male; Mice; Mice, Congenic; Mice, Inbred CFTR; Microsatellite Repeats; Phenotype | 2008 |
Activation of Cl- currents by intracellular chloride in fibroblasts stably expressing the human cystic fibrosis transmembrane conductance regulator.
Topics: Animals; Cells, Cultured; Chloride Channels; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Fibroblasts; Humans; Membrane Proteins; Mice; Neural Conduction | 1993 |
Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator.
Topics: 3T3 Cells; Animals; Bicarbonates; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Hydrogen-Ion Concentration; Ion Channel Gating; Membrane Proteins; Mice; Recombinant Proteins | 1994 |
Activation of the cystic fibrosis transmembrane regulator by cyclic AMP is not correlated with inhibition of endocytosis.
Topics: Cell Line; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dextrans; Dose-Response Relationship, Drug; Endocytosis; Fluorescein-5-isothiocyanate; Humans; Iodine; Membrane Proteins; Time Factors | 1994 |
Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR.
Topics: Animals; Base Sequence; Carrier Proteins; Chlorides; Colforsin; Colon; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Fatty Acid-Binding Protein 7; Fatty Acid-Binding Proteins; Gene Expression; Genetic Therapy; Humans; Intestinal Mucosa; Intestine, Small; Membrane Proteins; Mice; Mice, Transgenic; Molecular Sequence Data; Neoplasm Proteins; Nerve Tissue Proteins; Promoter Regions, Genetic; Rats; Recombinant Proteins; RNA, Messenger; Tumor Suppressor Proteins | 1994 |
CFTR displays voltage dependence and two gating modes during stimulation.
Topics: 3T3 Cells; Analysis of Variance; Animals; Chloride Channels; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Ion Channel Gating; Membrane Potentials; Membrane Proteins; Mice; Models, Biological; Patch-Clamp Techniques; Transfection | 1994 |
Effects of mutations in cAMP-dependent protein kinase on chloride efflux in Caco-2 human colonic carcinoma cells.
Topics: Carbachol; Chloride Channels; Colforsin; Colonic Neoplasms; Cyclic AMP; Cyclic AMP-Dependent Protein Kinases; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dinoprostone; Enzyme Activation; Humans; Isoproterenol; Membrane Proteins; Mutation; Second Messenger Systems; Transformation, Genetic; Tumor Cells, Cultured; Vasoactive Intestinal Peptide; Zinc | 1995 |
Increasing expression of the normal human CFTR cDNA in cystic fibrosis epithelial cells results in a progressive increase in the level of CFTR protein expression, but a limit on the level of cAMP-stimulated chloride secretion.
Topics: Adenocarcinoma; Adenoviruses, Human; Animals; Chlorides; Colforsin; Colonic Neoplasms; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA, Complementary; Epithelium; Genetic Vectors; Humans; Mammary Neoplasms, Experimental; Membrane Proteins; Mice; Pancreatic Neoplasms; Recombinant Fusion Proteins; Secretory Rate; Tumor Cells, Cultured | 1994 |
cAMP- but not Ca(2+)-regulated Cl- conductance in the oviduct is defective in mouse model of cystic fibrosis.
Topics: Adenosine Triphosphate; Animals; Blotting, Western; Calcium; Chloride Channels; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Fallopian Tubes; Female; Indomethacin; Ionomycin; Kinetics; Membrane Proteins; Mice | 1995 |
Ion transport across the jejunum in normal and cystic fibrosis mice.
Topics: Animals; Bicarbonates; Bumetanide; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Electric Conductivity; Female; Glucose; Ion Channels; Jejunum; Kinetics; Male; Mice; Monosaccharide Transport Proteins; Sodium; Tetrodotoxin | 1995 |
Forskolin stimulates swelling-induced chloride current, not cardiac cystic fibrosis transmembrane-conductance regulator current, in human cardiac myocytes.
Topics: Action Potentials; Adult; Aged; Chloride Channels; Colforsin; Cyclic AMP-Dependent Protein Kinases; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrophysiology; Female; Heart Atria; Heart Ventricles; Humans; In Vitro Techniques; Inflammation; Male; Membrane Proteins; Middle Aged; Myocardium; Patch-Clamp Techniques; Stimulation, Chemical | 1995 |
ATP-sensitive K+ channels regulated by intracellular Ca2+ and phosphorylation in normal (T84) and cystic fibrosis (CFPAC-1) epithelial cells.
Topics: Acetylcholine; Adenosine Triphosphate; Barium; Biotransformation; Calcium; Cell Line; Charybdotoxin; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Epithelium; Humans; Ionomycin; Membrane Proteins; Patch-Clamp Techniques; Phosphorylation; Potassium Channels; Scorpion Venoms | 1995 |
ATPo but not cAMPi activates a chloride conductance in mouse ventricular myocytes.
Topics: 1-Methyl-3-isobutylxanthine; Adenosine; Adenosine Triphosphate; Animals; Cells, Cultured; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Isoproterenol; Membrane Potentials; Mice; Mice, Knockout; Myocardium; Patch-Clamp Techniques; Stimulation, Chemical | 1995 |
Characterization of two distinct Cl- conductances in fused human respiratory epithelial cells. II. Relation to cystic fibrosis gene product.
Topics: Antibodies; Calcimycin; Calcium; Cell Fusion; Cells, Cultured; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Epithelium; Humans; Membrane Proteins; Nasal Polyps; Respiratory System | 1995 |
A powerful method for in vitro selection of normal versus cystic fibrosis airway epithelial cells.
Topics: Cell Line, Transformed; Cell Separation; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epinephrine; Epithelium; Gene Targeting; Genetic Therapy; Humans; In Vitro Techniques; Respiratory System | 1994 |
Characterization of two distinct Cl- conductances in fused human respiratory epithelial cells. I. Anion selectivities, stimulation and intermeshing signal transduction pathways.
Topics: Anions; Calcimycin; Carbachol; Cell Fusion; Cells, Cultured; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Electric Conductivity; Epithelium; Humans; Nasal Polyps; Respiratory System; Signal Transduction | 1995 |
Regulation of membrane chloride currents in rat bile duct epithelial cells.
Topics: 4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic Acid; 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; Animals; Bile Ducts; Calcium; Cells, Cultured; Chloride Channels; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Epithelium; Iodides; Ionomycin; Kinetics; Liver; Male; Membrane Potentials; Membrane Proteins; Rats; Rats, Sprague-Dawley; Thionucleotides | 1993 |
Cell surface labeling of CFTR in T84 cells.
Topics: Autoradiography; Biotin; Cell Line; Cell Membrane; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrophoresis, Polyacrylamide Gel; Humans; Membrane Proteins; Periodic Acid; Precipitin Tests | 1993 |
Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy.
Topics: Animals; Base Sequence; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Carriers; Epithelial Cells; Epithelium; Genetic Therapy; HeLa Cells; Humans; In Situ Hybridization; Ion Channels; Liposomes; Membrane Proteins; Mice; Mice, Transgenic; Oligonucleotide Probes; Plasmids; RNA, Messenger; Trachea; Transfection | 1993 |
Long-term cAMP activation of Na(+)-K(+)-2Cl- cotransporter activity in HT-29 human adenocarcinoma cells.
Topics: Adenocarcinoma; Bumetanide; Carrier Proteins; Chlorides; Colforsin; Colonic Neoplasms; Cyclic AMP; Cycloheximide; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dactinomycin; Humans; Kinetics; Membrane Proteins; Potassium; Protein Biosynthesis; Sodium; Sodium-Potassium-Chloride Symporters; Transcription, Genetic; Transfection; Tumor Cells, Cultured | 1993 |
Isobutylmethylxanthine fails to stimulate chloride secretion in cystic fibrosis airway epithelia.
Topics: 1-Methyl-3-isobutylxanthine; Adenylyl Cyclases; Adult; Amiloride; Bronchi; Cells, Cultured; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Electric Conductivity; Enzyme Activation; Epithelium; Female; Humans; Male | 1993 |
Ketoconazole activates Cl- conductance and blocks Cl- and fluid absorption by cultured cystic fibrosis (CFPAC-1) cells.
Topics: Absorption; Cell Line; Cell Membrane; Chloride Channels; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelium; Humans; Hydrogen-Ion Concentration; Indomethacin; Ion Channels; Ketoconazole; Masoprocol; Membrane Potentials; Membrane Proteins; Proadifen; Thionucleotides; Transfection | 1993 |
CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship.
Topics: 3T3 Cells; Animals; Cells, Cultured; Chloride Channels; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Ion Channels; Membrane Potentials; Membrane Proteins; Mice; Protein Kinases | 1993 |
Functional consequences of heterologous expression of the cystic fibrosis transmembrane conductance regulator in fibroblasts.
Topics: 3T3 Cells; 4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic Acid; 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; Animals; Blotting, Western; Chloride Channels; Chlorides; Clone Cells; Colforsin; Colonic Neoplasms; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Fibroblasts; Humans; Ion Channels; Membrane Potentials; Membrane Proteins; Mice; Recombinant Proteins; Transfection; Tumor Cells, Cultured | 1993 |
Glycosylation status of endogenous CFTR does not affect cAMP-stimulated Cl- secretion in epithelial cells.
Topics: Cell Line; Cell Polarity; Chlorides; Colforsin; Colon; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Fluorescent Antibody Technique; Glycosylation; Humans; Intestinal Mucosa; Membrane Proteins; Neurotensin; Oligosaccharides; Protein Processing, Post-Translational | 1993 |
The genetic advantage hypothesis in cystic fibrosis heterozygotes: a murine study.
Topics: Animals; Bacterial Toxins; Calcium; Carbachol; Chloride Channels; Cholera Toxin; Colforsin; Colon; Cyclic AMP; Cystic Fibrosis; Enterotoxins; Epithelium; Escherichia coli Proteins; Furosemide; Gastrointestinal Hormones; Glucose; Heterozygote; Ileum; Isoproterenol; Kallidin; Mice; Mice, Mutant Strains; Natriuretic Peptides; Peptides; Vasoactive Intestinal Peptide | 1995 |
Additional similarity of cardiac cAMP-activated Cl- channels to CFTR Cl- channels.
Topics: Animals; Blotting, Northern; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Glyburide; Guinea Pigs; Heart; RNA, Messenger | 1994 |
Molecular physiology of CFTR Cl- channels in heart.
Topics: Amino Acid Sequence; Animals; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Dogs; Guinea Pigs; Heart; Humans; Membrane Proteins; Molecular Sequence Data; Rabbits | 1994 |
Ion-transporting activity in the murine colonic epithelium of normal animals and animals with cystic fibrosis.
Topics: Amiloride; Animals; Biological Transport; Carbachol; Colforsin; Colon; Cystic Fibrosis; Epithelium; Furosemide; Ions; Mice; Mice, Inbred CFTR; Potassium; Rubidium Radioisotopes; Tetraethylammonium; Tetraethylammonium Compounds | 1994 |
Anomalies in ion transport in CF mouse tracheal epithelium.
Topics: Animals; Biological Transport; Calcium; Chlorides; Colforsin; Cystic Fibrosis; Electrophysiology; Epithelium; Female; Ionomycin; Male; Mice; Mice, Mutant Strains; Sodium; Trachea | 1994 |
Effects of sulphonylureas on cAMP-stimulated Cl- transport via the cystic fibrosis gene product in human epithelial cells.
Topics: Adenosine Triphosphate; Blood Proteins; Cell Line; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diazoxide; Electrophysiology; Glyburide; Humans; Sulfonylurea Compounds; Tolbutamide | 1994 |
The introduction of two silent mutations into a CFTR cDNA construct allows improved detection of exogenous mRNA in gene transfer experiments.
Topics: Base Sequence; Cell Line; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA, Complementary; Gene Transfer Techniques; Genetic Vectors; Humans; Ionomycin; Molecular Sequence Data; Mutagenesis; Nasal Mucosa; Transcription, Genetic | 1995 |
Swelling and Ca2+-activated anion conductances in C127 epithelial cells expressing WT and delta F508-CFTR.
Topics: Animals; Biological Transport, Active; Bovine papillomavirus 1; Calcium; Cell Line; Cell Size; Chloride Channels; Colforsin; Cystic Fibrosis; Dose-Response Relationship, Drug; Drug Interactions; Electric Conductivity; Female; Hypotonic Solutions; In Vitro Techniques; Ionomycin; Mice | 1996 |
Evidence against defective trans-Golgi acidification in cystic fibrosis.
Topics: 3T3 Cells; Animals; Bicarbonates; Cattle; Cell Compartmentation; Cells, Cultured; Chloride Channels; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Endosomes; Golgi Apparatus; Humans; Hydrogen-Ion Concentration; Mice; Mutation; Recombinant Proteins; Transfection | 1996 |
Quantitative fluorescence measurements of chloride secretion in native airway epithelium from CF and non-CF subjects.
Topics: Adult; Aged; Bronchi; Cells, Cultured; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelium; Fluorescent Dyes; Genetic Therapy; Humans; Ionomycin; Kinetics; Middle Aged; Nasal Mucosa; Quinolinium Compounds; Reference Values; Transfection; Turbinates | 1995 |
cAMP- but not Ca(2+)-regulated Cl- conductance is lacking in cystic fibrosis mice epididymides and seminal vesicles.
Topics: Adenosine Triphosphate; Animals; Calcium; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Electric Conductivity; Epididymis; Ionomycin; Male; Mice; Mice, Inbred C57BL; Reference Values; Seminal Vesicles | 1996 |
Defective ATP-dependent mucin secretion by cystic fibrosis pancreatic epithelial cells.
Topics: Adenocarcinoma; Adenosine; Adenosine Diphosphate; Adenosine Monophosphate; Adenosine Triphosphate; Adult; Antigens; Calcium; Carbachol; Cell Line; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelium; Humans; Immunoassay; Mucins; Pancreatic Neoplasms; Receptors, Purinergic P2; Transfection; Tumor Cells, Cultured; Vasoactive Intestinal Peptide | 1996 |
A demonstration using mouse models that successful gene therapy for cystic fibrosis requires only partial gene correction.
Topics: Animals; Chlorides; Colforsin; Crosses, Genetic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Gene Expression; Genes, Recessive; Genetic Therapy; Genotype; Intestines; Mice; Mice, Mutant Strains; Phenotype; RNA, Messenger | 1996 |
Cl- currents activated by extracellular nucleotides in human bronchial cells.
Topics: Adenosine Triphosphate; Bronchi; Calcium; Cells, Cultured; Chelating Agents; Chlorides; Colforsin; Cystic Fibrosis; Egtazic Acid; Epithelial Cells; Epithelium; Extracellular Space; Humans; Intracellular Fluid; Ion Transport; Membrane Potentials; Niflumic Acid; Patch-Clamp Techniques; Uridine Triphosphate | 1997 |
Incomplete rescue of cystic fibrosis transmembrane conductance regulator deficient mice by the human CFTR cDNA.
Topics: Alleles; Animals; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrophysiology; Homozygote; Humans; Intestines; Mice; Mice, Transgenic; Phenotype; Recombinant Proteins; Recombination, Genetic; Transgenes | 1997 |
Complementation of null CF mice with a human CFTR YAC transgene.
Topics: Animals; Carbachol; Chlorides; Chromosomes, Artificial, Yeast; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrophysiology; Furosemide; Gene Expression Regulation; Genetic Complementation Test; Humans; In Situ Hybridization; Intestinal Mucosa; Lung; Mice; Mice, Knockout; Mice, Transgenic; Pancreatic Ducts; RNA, Messenger; Salivary Glands; Transgenes | 1997 |
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.
Topics: Biological Transport; Cells, Cultured; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Immunohistochemistry; Kinetics; Membrane Potentials; Nasal Mucosa; Nasal Polyps; Patch-Clamp Techniques; Phenylbutyrates; Point Mutation | 1997 |
In vivo activation of CFTR-dependent chloride transport in murine airway epithelium by CNP.
Topics: 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; Amiloride; Animals; Anti-Inflammatory Agents, Non-Steroidal; Chlorides; Colforsin; Cyclic GMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Mice; Mice, Knockout; Mice, Mutant Strains; Models, Biological; Nasal Mucosa; Natriuretic Peptide, C-Type; Nitroprusside; ortho-Aminobenzoates; Proteins; Trachea | 1997 |
Ability of a chimeric cAMP-responsive promoter to confer pharmacologic control of CFTR cDNA expression and cAMP-mediated Cl- secretion.
Topics: Adenoviridae; Anions; Cell Line; Chlorides; Colforsin; Combined Modality Therapy; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA, Complementary; Gene Expression Regulation; Gene Transfer Techniques; Genetic Therapy; Genetic Vectors; Humans; Promoter Regions, Genetic | 1997 |
Effect of IBMX and alkaline phosphatase inhibitors on Cl- secretion in G551D cystic fibrosis mutant mice.
Topics: 1-Methyl-3-isobutylxanthine; Alkaline Phosphatase; Animals; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Jejunum; Levamisole; Mice; Mice, Mutant Strains; Phosphodiesterase Inhibitors; Rectum; Tetramisole; Trachea | 1998 |
The primary and final effector mechanisms required for kinin-induced epithelial chloride secretion.
Topics: Animals; Chloride Channels; Chlorides; Chromosomes, Artificial, Yeast; Colforsin; Cystic Fibrosis; Humans; Intestinal Mucosa; Kallidin; Mice; Mice, Knockout; Receptor, Bradykinin B2; Receptors, Bradykinin | 1998 |
Antibiotic accumulation and membrane trafficking in cystic fibrosis cells.
Topics: 1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine; 1-Methyl-3-isobutylxanthine; Adenosine Triphosphate; Adenylyl Imidodiphosphate; Adult; Cell Membrane; Child; Child, Preschool; Colforsin; Cyclic AMP; Cyclic AMP-Dependent Protein Kinases; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Energy Metabolism; Female; Fluorescein-5-isothiocyanate; Gentamicins; Humans; Male; Middle Aged; Nasal Polyps; Thionucleotides | 1998 |
Importance of basolateral K+ conductance in maintaining Cl- secretion in murine nasal and colonic epithelia.
Topics: Amiloride; Animals; Benzimidazoles; Calcium; Calcium Channel Agonists; Chlorides; Colforsin; Colon; Cystic Fibrosis; Electric Conductivity; Intestinal Mucosa; Intracellular Membranes; Mice; Nasal Mucosa; Osmolar Concentration; Potassium; Potassium Channel Blockers | 1998 |
Restoration by intratracheal gene transfer of bicarbonate secretion in cystic fibrosis mouse gallbladder.
Topics: Animals; beta-Galactosidase; Bicarbonates; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Gallbladder; Gene Expression; Gene Transfer Techniques; Humans; Ionomycin; Liposomes; Luciferases; Mice; Mice, Inbred CFTR; Polymerase Chain Reaction; RNA, Messenger; Trachea | 1998 |
Interrelationship between the Na+/glucose cotransporter and CFTR in Caco-2 cells: relevance to cystic fibrosis.
Topics: Biological Transport; Caco-2 Cells; Calcium Channel Blockers; Cell Differentiation; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Enzyme Inhibitors; Glucose; Glyburide; Glycoconjugates; Humans; Ion Channel Gating; Membrane Glycoproteins; Monosaccharide Transport Proteins; ortho-Aminobenzoates; Phlorhizin; Sodium; Sodium-Glucose Transporter 1; Thionucleotides | 1998 |
Purinoceptor activation of chloride transport in cystic fibrosis and CFTR-transfected pancreatic cell lines.
Topics: Adenosine Triphosphate; Cell Line; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Enzyme Inhibitors; Humans; Pancreas; Purinergic Agonists; Receptors, Purinergic; Thapsigargin; Transfection; Uridine Triphosphate | 1998 |
Cystic fibrosis transmembrane conductance regulator-associated ATP release is controlled by a chloride sensor.
Topics: 1-Methyl-3-isobutylxanthine; Adenosine Triphosphate; Animals; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Female; Gene Expression; Membrane Potentials; Oocytes; Xenopus laevis | 1998 |
Formal analysis of electrogenic sodium, potassium, chloride and bicarbonate transport in mouse colon epithelium.
Topics: Acetazolamide; Amiloride; Animals; Bicarbonates; Biological Transport; Chlorides; Colforsin; Colon; Cystic Fibrosis; Diuretics; Electrophysiology; Epithelium; Furosemide; In Vitro Techniques; Inorganic Chemicals; Membrane Potentials; Mice; Mice, Inbred CFTR; Models, Theoretical; Potassium; Sodium | 1999 |
Activation of wild-type and deltaF508-CFTR by phosphodiesterase inhibitors through cAMP-dependent and -independent mechanisms.
Topics: 1-Methyl-3-isobutylxanthine; 3T3 Cells; Animals; Cell Line; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Mice; Milrinone; Mutation; Patch-Clamp Techniques; Phosphodiesterase Inhibitors; Thionucleotides; Xanthines | 1999 |
Synergistic effects of cAMP- and calcium-mediated amylase secretion in isolated pancreatic acini from cystic fibrosis mice.
Topics: 8-Bromo Cyclic Adenosine Monophosphate; Amylases; Animals; Body Weight; Calcium; Carbachol; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diet; Exocytosis; In Vitro Techniques; Mice; Mice, Knockout; Organ Size; Pancreas | 1999 |
Ion transport across the normal and CF neonatal murine intestine.
Topics: Amiloride; Amino Acid Transport Systems; Amino Acid Transport Systems, Neutral; Animals; Animals, Newborn; Carbachol; Carrier Proteins; Chlorides; Colforsin; Cystic Fibrosis; Electrochemistry; Electrophysiology; Intestinal Absorption; Intestinal Mucosa; Intestines; Ion Transport; Membrane Glycoproteins; Mice; Mice, Inbred Strains; Monosaccharide Transport Proteins; Reference Values; Sodium; Sodium-Glucose Transporter 1; Symporters | 1999 |
CFTR upregulates the expression of the basolateral Na(+)-K(+)-2Cl(-) cotransporter in cultured pancreatic duct cells.
Topics: Biological Transport; Carrier Proteins; Cells, Cultured; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Gene Expression; Humans; Pancreatic Ducts; Quaternary Ammonium Compounds; RNA, Messenger; Sodium; Sodium Bicarbonate; Sodium-Potassium-Chloride Symporters | 1999 |
Stimulation of Cl(-) secretion by chlorzoxazone.
Topics: Amiloride; Anions; Bronchi; Bumetanide; Cell Membrane; Cells, Cultured; Charybdotoxin; Chlorine; Chlorzoxazone; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Interactions; Epithelium; Glyburide; Humans; Isoproterenol; Nasal Mucosa; Nystatin; Potassium Channel Blockers; Rubidium; Zoxazolamine | 2000 |
Novel Cystic Fibrosis mutation L1093P: functional analysis and possible Native American origin.
Topics: Adult; Anions; Base Sequence; Cells, Cultured; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indians, North American; Mutagenesis, Site-Directed; Mutation, Missense; Pedigree; Polymerase Chain Reaction; Polymorphism, Single-Stranded Conformational; Transfection | 2000 |
Deletion of phenylalanine 508 causes attenuated phosphorylation-dependent activation of CFTR chloride channels.
Topics: 3T3 Cells; Adenosine Triphosphate; Animals; Colforsin; Cyclic AMP; Cyclic AMP-Dependent Protein Kinases; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Enzyme Inhibitors; Gene Deletion; Genistein; Ion Channel Gating; Mice; Patch-Clamp Techniques; Phenylalanine; Phosphorylation; Point Mutation; Protein Structure, Tertiary; Thionucleotides | 2000 |
Cystic fibrosis transmembrane conductance regulator currents in guinea pig pancreatic duct cells: inhibition by bicarbonate ions.
Topics: Animals; Anions; Bicarbonates; Carbon Dioxide; Chloride Channels; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Electrophysiology; Female; Guinea Pigs; Hydrogen-Ion Concentration; Male; Membrane Potentials; Pancreatic Ducts; Pancreatic Juice; Secretin; Sodium Bicarbonate | 2000 |
Extracellular UTP stimulates electrogenic bicarbonate secretion across CFTR knockout gallbladder epithelium.
Topics: 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; Age Factors; Animals; Bicarbonates; Biological Transport; Calcium; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Electrophysiology; Epithelial Cells; Gallbladder; Hydrogen-Ion Concentration; In Vitro Techniques; Membrane Potentials; Mice; Mice, Inbred C57BL; Mice, Knockout; Purinergic P2 Receptor Agonists; Receptors, Purinergic P2; Receptors, Purinergic P2Y2; Stimulation, Chemical; Uridine Triphosphate | 2000 |
Effect of genistein on native epithelial tissue from normal individuals and CF patients and on ion channels expressed in Xenopus oocytes.
Topics: 1-Methyl-3-isobutylxanthine; Adolescent; Adult; Aged; Amiloride; Animals; Child; Child, Preschool; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Epithelium; Female; Gene Expression; Genistein; Humans; Ion Channels; Ion Transport; Membrane Potentials; Middle Aged; Mutation; Nasal Cavity; Oocytes; Rectum; Sodium Channels; Xenopus | 2000 |
Role of K(V)LQT1 in cyclic adenosine monophosphate-mediated Cl(-) secretion in human airway epithelia.
Topics: 1-Methyl-3-isobutylxanthine; Amiloride; Biological Transport; Bronchi; Cell Line, Transformed; Chlorides; Chromans; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diuretics; Gene Expression; Humans; KCNQ Potassium Channels; KCNQ1 Potassium Channel; Membrane Potentials; Patch-Clamp Techniques; Phosphodiesterase Inhibitors; Potassium; Potassium Channel Blockers; Potassium Channels; Potassium Channels, Voltage-Gated; Respiratory Mucosa; Sulfonamides | 2000 |
CFTR regulation of intracellular calcium in normal and cystic fibrosis human airway epithelia.
Topics: Adenosine Triphosphate; Calcium; Cell Line; Cells, Cultured; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Glyburide; Hexokinase; Humans; Intracellular Fluid; Lung; Mutation; Nucleotides; ortho-Aminobenzoates; Receptors, Purinergic; Suramin; Temperature | 2000 |
Improved oxygenation promotes CFTR maturation and trafficking in MDCK monolayers.
Topics: Animals; Cell Culture Techniques; Cell Differentiation; Cell Membrane; Cell Polarity; Cells, Cultured; Colforsin; Culture Media; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dogs; Hypoxia; Oxygen; Protein Transport; RNA, Messenger | 2001 |
Assessment of CFTR chloride channel openers in intact normal and cystic fibrosis murine epithelia.
Topics: Animals; Benzimidazoles; Calcium Channel Blockers; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Electric Conductivity; Intestinal Mucosa; Mice | 2001 |
ClC-2 Cl- channels in human lung epithelia: activation by arachidonic acid, amidation, and acid-activated omeprazole.
Topics: 1-Methyl-3-isobutylxanthine; Adenosine Triphosphate; Amides; Arachidonic Acids; Cell Line; Cells, Cultured; Chloride Channels; Chlorides; CLC-2 Chloride Channels; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Enzyme Inhibitors; Ethyldimethylaminopropyl Carbodiimide; Humans; Magnesium; Mouth Mucosa; Omeprazole; Patch-Clamp Techniques; Phosphodiesterase Inhibitors; Recombinant Proteins; Respiratory Mucosa | 2001 |
Partial restoration of defective chloride conductance in DeltaF508 CF mice by trimethylamine oxide.
Topics: Animals; Apigenin; Chloride Channels; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Electric Conductivity; Electrophysiology; Flavonoids; Glyburide; Methylamines; Mice; Oxidants; Rectum; Reference Values | 2001 |
Evidence that CFTR channels can regulate the open duration of other CFTR channels: cooperativity.
Topics: Cell Line; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Ion Channel Gating; Mathematics; Patch-Clamp Techniques; Respiratory Mucosa | 2001 |
Assessment of chloride secretion in human nasal epithelial cells by X-ray microanalysis.
Topics: 1-Methyl-3-isobutylxanthine; Adenosine Triphosphate; Chlorides; Colforsin; Cystic Fibrosis; Electron Probe Microanalysis; Epithelial Cells; Humans; Ion Transport; Nasal Mucosa | 2001 |
Examining basal chloride transport using the nasal potential difference response in a murine model.
Topics: 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; Amiloride; Animals; Chloride Channels; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Female; Humans; Ion Transport; Membrane Potentials; Mice; Mice, Inbred Strains; Nasal Mucosa; Thionucleotides | 2001 |
CFTR and lysozyme secretion in human airway epithelial cells.
Topics: 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; Biological Transport; Cell Line; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Enzyme Inhibitors; Humans; Muramidase; Nitric Oxide Donors; Oligonucleotides, Antisense; Respiratory Mucosa; S-Nitrosoglutathione; Thapsigargin | 2001 |
Non-CFTR chloride channels likely contribute to secretion in the murine small intestine.
Topics: Angiogenesis Inhibitors; Animals; Biological Transport; Calcium Signaling; Chloride Channels; Chlorides; CLC-2 Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Gene Expression; Ileum; Mice; Mice, Inbred CFTR; Nitrobenzoates; RNA, Messenger | 2001 |
EBIO, an agent causing maintained epithelial chloride secretion by co-ordinate actions at both apical and basolateral membranes.
Topics: Animals; Benzimidazoles; Calcium Channel Agonists; Cell Polarity; Charybdotoxin; Chlorides; Chlorzoxazone; Colforsin; Colon; Cyclic AMP; Cystic Fibrosis; Dose-Response Relationship, Drug; In Vitro Techniques; Intestinal Mucosa; Mice; Muscle Relaxants, Central; Nasal Mucosa; Potassium Channels | 2001 |
Gene complementation of airway epithelium in the cystic fibrosis mouse is necessary and sufficient to correct the pathogen clearance and inflammatory abnormalities.
Topics: 1-Methyl-3-isobutylxanthine; Animals; Bronchoalveolar Lavage; Cells, Cultured; Chemokine CXCL2; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrophysiology; Enzyme-Linked Immunosorbent Assay; Epithelial Cells; Female; Fluorescent Antibody Technique; Genetic Complementation Test; Humans; Lung; Macrophages, Alveolar; Male; Mice; Mice, Inbred C57BL; Mice, Transgenic; Monokines; Mutation; Phosphodiesterase Inhibitors; Pseudomonas aeruginosa; Pseudomonas Infections; RNA, Messenger; Tumor Necrosis Factor-alpha | 2002 |
CFTR channels in immortalized human airway cells.
Topics: Anions; Bronchi; Cell Line, Transformed; Chloride Channels; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Humans; Hypotonic Solutions; Iodides; Ion Channels; Ionomycin; Membrane Proteins | 1992 |
Reversed anion selectivity in cultured cystic fibrosis sweat duct cells.
Topics: Anions; Cell Division; Cells, Cultured; Chloride Channels; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Electric Conductivity; Humans; Iodides; Membrane Potentials; Membrane Proteins; Phenotype; Reference Values; Sweat Glands | 1992 |
cAMP stimulates bicarbonate secretion across normal, but not cystic fibrosis airway epithelia.
Topics: Animals; Bicarbonates; Cells, Cultured; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Dogs; Epithelium; Humans; Nasal Mucosa; Sodium; Trachea | 1992 |
Ion transport characteristics of the murine trachea and caecum.
Topics: Amiloride; Animals; Bumetanide; Cecum; Chlorides; Colforsin; Cystic Fibrosis; Electric Conductivity; In Vitro Techniques; Male; Mice; Mice, Transgenic; Phorbol 12,13-Dibutyrate; Pyridazines; Sodium; Trachea | 1992 |
Cellular differentiation is required for cAMP but not Ca(2+)-dependent Cl- secretion in colonic epithelial cells expressing high levels of cystic fibrosis transmembrane conductance regulator.
Topics: Adenocarcinoma; Calcium; Cell Differentiation; Cell Line; Chloride Channels; Chlorides; Colforsin; Colonic Neoplasms; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Epithelium; Humans; Ion Channels; Ionomycin; Kinetics; Membrane Potentials; Membrane Proteins; Microscopy, Electron; Neurotensin; Poly A; RNA, Messenger | 1992 |
T84 cells: anion selectivity demonstrates expression of Cl- conductance affected in cystic fibrosis.
Topics: Amiloride; Anions; Azides; Bumetanide; Chlorides; Colforsin; Colonic Neoplasms; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Epithelium; Humans; Kinetics; Membrane Potentials; Membrane Proteins; Potassium; Sodium Azide; Tumor Cells, Cultured | 1992 |
cAMP-stimulated ion currents in Xenopus oocytes expressing CFTR cRNA.
Topics: 4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic Acid; 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; Alkaline Phosphatase; Animals; Calcium; Cells, Cultured; Chlorides; Cloning, Molecular; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Membrane Potentials; Membrane Proteins; Oocytes; RNA; Transcription, Genetic; Xenopus laevis | 1992 |
Role of CFTR in lysosome acidification.
Topics: Adenocarcinoma; Adenosine Triphosphate; Bucladesine; Carbonyl Cyanide m-Chlorophenyl Hydrazone; Cell Line; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dextrans; Fluorescein-5-isothiocyanate; Fluorescent Dyes; Hydrogen-Ion Concentration; Kinetics; Lysosomes; Membrane Proteins; Monensin; Mutation; Pancreatic Neoplasms; Spectrometry, Fluorescence | 1992 |
Regulation of plasma membrane recycling by CFTR.
Topics: Base Sequence; Cell Membrane; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA; Endocytosis; Epithelium; Exocytosis; Gene Expression; Horseradish Peroxidase; Humans; Membrane Proteins; Molecular Sequence Data; Pancreatic Neoplasms; Transfection; Tumor Cells, Cultured; Wheat Germ Agglutinins | 1992 |
Induction of expression of the cystic fibrosis transmembrane conductance regulator.
Topics: Adenocarcinoma; Amanitins; Blotting, Western; Chlorides; Colforsin; Cyclic AMP; Cycloheximide; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dactinomycin; DNA; Humans; Membrane Proteins; Promoter Regions, Genetic; Protein Biosynthesis; Tetradecanoylphorbol Acetate; Transcription, Genetic; Tumor Cells, Cultured; Up-Regulation | 1992 |
The cystic fibrosis transmembrane regulator is present and functional in endosomes. Role as a determinant of endosomal pH.
Topics: Alkaline Phosphatase; Animals; Biological Transport; Cell Membrane Permeability; Chlorides; CHO Cells; Colforsin; Cricetinae; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Hydrogen-Ion Concentration; Intracellular Membranes; Membrane Proteins; Phosphorylation; Plasmids; Protein Kinase Inhibitors; Protein Kinases; Transfection; Vacuoles | 1992 |
Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis.
Topics: Amiloride; Animals; Biological Transport; Cells, Cultured; Chlorides; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Epithelium; Intestinal Mucosa; Membrane Proteins; Mice; Mutation; Nasal Mucosa; Trachea | 1992 |
Regulated membrane vesicle trafficking: a defect in cystic fibrosis corrected by gene transfer.
Topics: Cell Line; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Endocytosis; Genetic Vectors; Humans; Membrane Proteins; Transfection | 1992 |
Ion transport in cultured epithelia from human sweat glands: comparison of normal and cystic fibrosis tissues.
Topics: Adenylyl Cyclases; Amiloride; Barium; Calcium; Carbachol; Cells, Cultured; Colforsin; Cyclic AMP; Cystic Fibrosis; Epithelium; Humans; Kallidin; Lanthanum; Sodium Channels; Sweat Glands; Terpenes; Thapsigargin | 1991 |
Chloride permeability regulation via a cyclic AMP pathway in cultured human sweat duct cells.
Topics: 1-Methyl-3-isobutylxanthine; 8-Bromo Cyclic Adenosine Monophosphate; Adrenergic Agonists; Cell Membrane Permeability; Cells, Cultured; Chlorides; Cholera Toxin; Colforsin; Cyclic AMP; Cystic Fibrosis; Dinoprostone; Humans; Membrane Potentials; Sweat Glands | 1990 |
Genetic complementation in cystic fibrosis pancreatic cells by somatic cell fusion.
Topics: Animals; Base Sequence; Biological Transport; Bromides; Cell Fusion; Cell Line; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Complementation Test; Humans; Membrane Proteins; Mice; Mice, Inbred Strains; Molecular Sequence Data; Oligonucleotide Probes | 1990 |
Expression of normal and cystic fibrosis phenotypes by continuous airway epithelial cell lines.
Topics: Antigens, Polyomavirus Transforming; Base Sequence; Biomarkers; Blood Proteins; Bradykinin; Calcimycin; Calgranulin A; Cell Line; Cell Transformation, Neoplastic; Cells, Cultured; Chloride Channels; Chlorides; Colforsin; Cystic Fibrosis; Epithelial Cells; Epithelium; Genotype; Humans; Ion Channels; Keratins; Membrane Potentials; Membrane Proteins; Molecular Sequence Data; Nasal Mucosa; Oligonucleotide Probes; Phenotype; Polymerase Chain Reaction; Reference Values; Retroviridae; Simian virus 40; Tetradecanoylphorbol Acetate; Theophylline | 1990 |
Functional insertion of the SV40 large T oncogene in cystic fibrosis intestinal epithelium. Characterization of CFI-3 cells.
Topics: Antigens, Polyomavirus Transforming; Base Sequence; Cell Line; Cell Transformation, Viral; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Gene Expression; Humans; In Vitro Techniques; Intestinal Mucosa; Ionomycin; Isoproterenol; Membrane Proteins; Molecular Sequence Data; Neuropeptides; Pituitary Adenylate Cyclase-Activating Polypeptide; Potassium Channels; Transfection; Vasoactive Intestinal Peptide | 1991 |
Further evidence for abnormal protein kinase C regulation of macromolecule secretion in fibroblasts from cystic fibrosis patients.
Topics: Binding Sites; Bucladesine; Calcimycin; Cells, Cultured; Colforsin; Cystic Fibrosis; Dose-Response Relationship, Drug; Fibroblasts; Glycoconjugates; Humans; Isoproterenol; Phorbol 12,13-Dibutyrate; Protein Kinase C; Skin; Tetradecanoylphorbol Acetate | 1990 |
Down-regulation of cystic fibrosis gene mRNA transcript levels and induction of the cystic fibrosis chloride secretory phenotype in epithelial cells by phorbol ester.
Topics: Blotting, Northern; Chlorides; Colforsin; Cystic Fibrosis; Down-Regulation; Epithelial Cells; Epithelium; Humans; Phenotype; RNA, Messenger; Tetradecanoylphorbol Acetate; Transcription, Genetic; Tumor Cells, Cultured | 1991 |
Persistence of abnormal chloride conductance regulation in transformed cystic fibrosis epithelia.
Topics: Amiloride; Antigens, Polyomavirus Transforming; Calcimycin; Cell Line; Cell Membrane; Chloride Channels; Chlorides; Colforsin; Cystic Fibrosis; Electric Conductivity; Epithelium; Ethers; Freeze Fracturing; Humans; Intercellular Junctions; Ion Channels; Ionomycin; Membrane Proteins; Microscopy, Electron; Nasal Polyps; Simian virus 40; Transformation, Genetic | 1989 |
Effect of cyclic adenosine monophosphate elevations on functional responses of polymorphonuclear leukocytes from patients with cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Colforsin; Cyclic AMP; Cystic Fibrosis; Cytoplasmic Granules; Epinephrine; Humans; Membrane Potentials; Neutrophils; Superoxides; Theophylline | 1989 |
The reserpine-treated rat as an experimental animal model for cystic fibrosis: abnormal Cl transport in pancreatic acinar cells.
Topics: 4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic Acid; 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; Acetylcholine; Amiloride; Animals; Biological Transport; Ceruletide; Chlorides; Colforsin; Cystic Fibrosis; Disease Models, Animal; Furosemide; Kinetics; Male; Pancreas; Rats; Rats, Inbred Strains; Reserpine; Vasoactive Intestinal Peptide | 1988 |