Note to users. If you're seeing this message, it means that your browser cannot find this page's style/presentation instructions -- or possibly that you are using a browser that does not support current Web standards. Find out more about why this message is appearing, and what you can do to make your experience of our site the best it can be.

Site Tools

  • AAAS
  • Subscribe
  • Feedback

Site Search

Search Advanced

Science 20 December 1991:
Vol. 254. no. 5039, pp. 1797 - 1799
DOI: 10.1126/science.1722350
Prev | Table of Contents | Next

Articles

Science, Vol 254, Issue 5039, 1797-1799
Copyright © 1991 by American Association for the Advancement of Science

articles

Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes

ML Drumm, DJ Wilkinson, LS Smit, RT Worrell, TV Strong, RA Frizzell, DC Dawson, and FS Collins

Department of Human Genetics, University of Michigan, Ann Arbor 48109.

The cystic fibrosis transmembrane conductance regulator (CFTR) is associated with expression of a chloride conductance that is defective in cystic fibrosis (CF). Xenopus oocytes injected with RNA coding for CFTR that contained mutations in the first nucleotide binding fold (NBF1) expressed chloride currents in response to raising adenosine 3',5'-monophosphate (cAMP) with forskolin and 3-isobutyl-1-methylxanthine (IBMX). The mutant CFTRs were less sensitive than wild-type CFTR to this activating stimulus, and the reduction in sensitivity correlated with the severity of cystic fibrosis in patients carrying the corresponding mutations. This demonstration provides the basis for detailed analyses of NBF1 function and suggests potential pharmacologic treatments for cystic fibrosis.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Role of N-linked oligosaccharides in the biosynthetic processing of the cystic fibrosis membrane conductance regulator.
X.-b. Chang, A. Mengos, Y.-x. Hou, L. Cui, T. J. Jensen, A. Aleksandrov, J. R. Riordan, and M. Gentzsch (2008)
J. Cell Sci. 121, 2814-2823
   Abstract »    Full Text »    PDF »
CLC-0 and CFTR: Chloride Channels Evolved From Transporters.
T.-Y. Chen and T.-C. Hwang (2008)
Physiol Rev 88, 351-387
   Abstract »    Full Text »    PDF »
Preclinical Evidence that Sildenafil and Vardenafil Activate Chloride Transport in Cystic Fibrosis.
B. Lubamba, H. Lecourt, J. Lebacq, P. Lebecque, H. De Jonge, P. Wallemacq, and T. Leal (2008)
Am. J. Respir. Crit. Care Med. 177, 506-515
   Abstract »    Full Text »    PDF »
Mechanism of G551D-CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) Potentiation by a High Affinity ATP Analog.
S. G. Bompadre, M. Li, and T.-C. Hwang (2008)
J. Biol. Chem. 283, 5364-5369
   Abstract »    Full Text »    PDF »
Structural Analog of Sildenafil Identified as a Novel Corrector of the F508del-CFTR Trafficking Defect.
R. Robert, G. W. Carlile, C. Pavel, N. Liu, S. M. Anjos, J. Liao, Y. Luo, D. Zhang, D. Y. Thomas, and J. W. Hanrahan (2008)
Mol. Pharmacol. 73, 478-489
   Abstract »    Full Text »    PDF »
Inhibiting Endoplasmic Reticulum (ER)-associated Degradation of Misfolded Yor1p Does Not Permit ER Export Despite the Presence of a Diacidic Sorting Signal.
S. Pagant, L. Kung, M. Dorrington, M. C.S. Lee, and E. A. Miller (2007)
Mol. Biol. Cell 18, 3398-3413
   Abstract »    Full Text »    PDF »
Inhibition of CFTR Cl- channel function caused by enzymatic hydrolysis of sphingomyelin.
Y. Ramu, Y. Xu, and Z. Lu (2007)
PNAS 104, 6448-6453
   Abstract »    Full Text »    PDF »
G551D and G1349D, Two CF-associated Mutations in the Signature Sequences of CFTR, Exhibit Distinct Gating Defects.
S. G. Bompadre, Y. Sohma, M. Li, and T.-C. Hwang (2007)
J. Gen. Physiol. 129, 285-298
   Abstract »    Full Text »    PDF »
Quantitation of protein kinase A-mediated trafficking of cardiac sodium channels in living cells.
H. Hallaq, Z. Yang, P. C. Viswanathan, K. Fukuda, W. Shen, D. W. Wang, K. S. Wells, J. Zhou, J. Yi, and K. T. Murray (2006)
Cardiovasc Res 72, 250-261
   Abstract »    Full Text »    PDF »
S-Nitrosylating Agents: A Novel Class of Compounds That Increase Cystic Fibrosis Transmembrane Conductance Regulator Expression and Maturation in Epithelial Cells.
K. Zaman, S. Carraro, J. Doherty, E. M. Henderson, E. Lendermon, L. Liu, G. Verghese, M. Zigler, M. Ross, E. Park, et al. (2006)
Mol. Pharmacol. 70, 1435-1442
   Abstract »    Full Text »    PDF »
Kir6.2 Mutations Associated With Neonatal Diabetes Reduce Expression of ATP-Sensitive K+ channels: Implications in Disease Mechanism and Sulfonylurea Therapy.
C.-W. Lin, Y.-W. Lin, F.-F. Yan, J. Casey, M. Kochhar, E. B. Pratt, and S.-L. Shyng (2006)
Diabetes 55, 1738-1746
   Abstract »    Full Text »    PDF »
Calreticulin Negatively Regulates the Cell Surface Expression of Cystic Fibrosis Transmembrane Conductance Regulator.
K. Harada, T. Okiyoneda, Y. Hashimoto, K. Ueno, K. Nakamura, K. Yamahira, T. Sugahara, T. Shuto, I. Wada, M. A. Suico, et al. (2006)
J. Biol. Chem. 281, 12841-12848
   Abstract »    Full Text »    PDF »
SERCA Pump Inhibitors Do Not Correct Biosynthetic Arrest of {Delta}F508 CFTR in Cystic Fibrosis.
B. R. Grubb, S. E. Gabriel, A. Mengos, M. Gentzsch, S. H. Randell, A. M. Van Heeckeren, M. R. Knowles, M. L. Drumm, J. R. Riordan, and R. C. Boucher (2006)
Am. J. Respir. Cell Mol. Biol. 34, 355-363
   Abstract »    Full Text »    PDF »
Pseudomonas aeruginosa inhibits endocytic recycling of CFTR in polarized human airway epithelial cells.
A. Swiatecka-Urban, S. Moreau-Marquis, D. P. MacEachran, J. P. Connolly, C. R. Stanton, J. R. Su, R. Barnaby, G. A. O'Toole, and B. A. Stanton (2006)
Am J Physiol Cell Physiol 290, C862-C872
   Abstract »    Full Text »    PDF »
An Energy-dependent Maturation Step Is Required for Release of the Cystic Fibrosis Transmembrane Conductance Regulator from Early Endoplasmic Reticulum Biosynthetic Machinery.
J. Oberdorf, D. Pitonzo, and W. R. Skach (2005)
J. Biol. Chem. 280, 38193-38202
   Abstract »    Full Text »    PDF »
The Short Apical Membrane Half-life of Rescued {Delta}F508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Results from Accelerated Endocytosis of {Delta}F508-CFTR in Polarized Human Airway Epithelial Cells.
A. Swiatecka-Urban, A. Brown, S. Moreau-Marquis, J. Renuka, B. Coutermarsh, R. Barnaby, K. H. Karlson, T. R. Flotte, M. Fukuda, G. M. Langford, et al. (2005)
J. Biol. Chem. 280, 36762-36772
   Abstract »    Full Text »    PDF »
Abnormal KCNQ1 trafficking influences disease pathogenesis in hereditary long QT syndromes (LQT1).
A. J. Wilson, K. V. Quinn, F. M. Graves, M. Bitner-Glindzicz, and A. Tinker (2005)
Cardiovasc Res 67, 476-486
   Abstract »    Full Text »    PDF »
Phenylglycine and Sulfonamide Correctors of Defective {Delta}F508 and G551D Cystic Fibrosis Transmembrane Conductance Regulator Chloride-Channel Gating.
N. Pedemonte, N. D. Sonawane, A. Taddei, J. Hu, O. Zegarra-Moran, Y. F. Suen, L. I. Robins, C. W. Dicus, D. Willenbring, M. H. Nantz, et al. (2005)
Mol. Pharmacol. 67, 1797-1807
   Abstract »    Full Text »    PDF »
Functional Comparison of Mouse slc26a6 Anion Exchanger with Human SLC26A6 Polypeptide Variants: DIFFERENCES IN ANION SELECTIVITY, REGULATION, AND ELECTROGENICITY.
M. N. Chernova, L. Jiang, D. J. Friedman, R. B. Darman, H. Lohi, J. Kere, D. H. Vandorpe, and S. L. Alper (2005)
J. Biol. Chem. 280, 8564-8580
   Abstract »    Full Text »    PDF »
Fukutin-related protein mutations that cause congenital muscular dystrophy result in ER-retention of the mutant protein in cultured cells.
C. T. Esapa, R.A. J. McIlhinney, and D. J. Blake (2005)
Hum. Mol. Genet. 14, 295-305
   Abstract »    Full Text »    PDF »
Modulation of {Delta}F508 Cystic Fibrosis Transmembrane Regulator Trafficking and Function with 4-Phenylbutyrate and Flavonoids.
M. Lim, K. McKenzie, A. D. Floyd, E. Kwon, and P. L. Zeitlin (2004)
Am. J. Respir. Cell Mol. Biol. 31, 351-357
   Abstract »    Full Text »    PDF »
{Delta}F508 CFTR Pool in the Endoplasmic Reticulum Is Increased by Calnexin Overexpression.
T. Okiyoneda, K. Harada, M. Takeya, K. Yamahira, I. Wada, T. Shuto, M. A. Suico, Y. Hashimoto, and H. Kai (2004)
Mol. Biol. Cell 15, 563-574
   Abstract »    Full Text »    PDF »
New concepts of the pathogenesis of cystic fibrosis lung disease.
R.C. Boucher (2004)
Eur. Respir. J. 23, 146-158
   Abstract »    Full Text »    PDF »
Organic Solutes Rescue the Functional Defect in {Delta}F508 Cystic Fibrosis Transmembrane Conductance Regulator.
X.-M. Zhang, X.-T. Wang, H. Yue, S. W. Leung, P. H. Thibodeau, P. J. Thomas, and S. E. Guggino (2003)
J. Biol. Chem. 278, 51232-51242
   Abstract »    Full Text »    PDF »
Nanomolar Affinity Small Molecule Correctors of Defective {Delta}F508-CFTR Chloride Channel Gating.
H. Yang, A. A. Shelat, R. K. Guy, V. S. Gopinath, T. Ma, K. Du, G. L. Lukacs, A. Taddei, C. Folli, N. Pedemonte, et al. (2003)
J. Biol. Chem. 278, 35079-35085
   Abstract »    Full Text »    PDF »
Mutations in the Nucleotide Binding Domain 1 Signature Motif Region Rescue Processing and Functional Defects of Cystic Fibrosis Transmembrane Conductance Regulator Delta F508.
A. C. V. deCarvalho, L. J. Gansheroff, and J. L. Teem (2002)
J. Biol. Chem. 277, 35896-35905
   Abstract »    Full Text »    PDF »
Phosphorylation and Putative ER Retention Signals Are Required for Protein Kinase A-Mediated Potentiation of Cardiac Sodium Current.
J. Zhou, H.-G. Shin, J. Yi, W. Shen, C. P. Williams, and K. T. Murray (2002)
Circ. Res. 91, 540-546
   Abstract »    Full Text »    PDF »
Cell-Biologic and Functional Analyses of Five New Aquaporin-2 Missense Mutations that Cause Recessive Nephrogenic Diabetes Insipidus.
N. Marr, D. G. Bichet, S. Hoefs, P. J. M. Savelkoul, I. B. M. Konings, F. de Mattia, M. P. J. Graat, M.-F. Arthus, M. Lonergan, T. M. Fujiwara, et al. (2002)
J. Am. Soc. Nephrol. 13, 2267-2277
   Abstract »    Full Text »    PDF »
KATP channels and insulin secretion disorders.
H. Huopio, S.-L. Shyng, T. Otonkoski, and C. G. Nichols (2002)
Am J Physiol Endocrinol Metab 283, E207-E216
   Abstract »    Full Text »    PDF »
Genistein Restores Functional Interactions between Delta F508-CFTR and ENaC in Xenopus Oocytes.
L. Suaud, J. Li, Q. Jiang, R. C. Rubenstein, and T. R. Kleyman (2002)
J. Biol. Chem. 277, 8928-8933
   Abstract »    Full Text »    PDF »
The severe G480C cystic fibrosis mutation, when replicated in the mouse, demonstrates mistrafficking, normal survival and organ-specific bioelectrics.
P. Dickinson, S. N. Smith, S. Webb, F. M. Kilanowski, I. J. Campbell, M. S. Taylor, D. J. Porteous, R. Willemsen, H. R. de Jonge, R. Farley, et al. (2002)
Hum. Mol. Genet. 11, 243-251
   Abstract »    Full Text »    PDF »
Activation of a CFTR-Mediated Chloride Current in a Rabbit Corneal Epithelial Cell Line.
L. Al-Nakkash and P. S. Reinach (2001)
Invest. Ophthalmol. Vis. Sci. 42, 2364-2370
   Abstract »    Full Text »    PDF »
A spectrum of functional effects for disease causing mutations in the Jervell and Lange-Nielsen syndrome.
L. Huang, M. Bitner-Glindzicz, L. Tranebjaerg, and A. Tinker (2001)
Cardiovasc Res 51, 670-680
   Abstract »    Full Text »    PDF »
Chaperoning the maturation of the cystic fibrosis transmembrane conductance regulator.
J. L. Brodsky (2001)
Am J Physiol Lung Cell Mol Physiol 281, L39-L42
   Full Text »    PDF »
Partial restoration of defective chloride conductance in {Delta}F508 CF mice by trimethylamine oxide.
H. Fischer, N. Fukuda, P. Barbry, B. Illek, C. Sartori, and M. A. Matthay (2001)
Am J Physiol Lung Cell Mol Physiol 281, L52-L57
   Abstract »    Full Text »    PDF »
Induction of HSP70 promotes {Delta}F508 CFTR trafficking.
L. R. Choo-Kang and P. L. Zeitlin (2001)
Am J Physiol Lung Cell Mol Physiol 281, L58-L68
   Abstract »    Full Text »    PDF »
Pharmacological treatment of the biochemical defect in cystic fibrosis airways.
H.C. Rodgers and A.J. Knox (2001)
Eur. Respir. J. 17, 1314-1321
   Abstract »    Full Text »    PDF »
Increased functional cell surface expression of CFTR and {Delta}F508-CFTR by the anthracycline doxorubicin.
R. Maitra, C. M. Shaw, B. A. Stanton, and J. W. Hamilton (2001)
Am J Physiol Cell Physiol 280, C1031-C1037
   Abstract »    Full Text »    PDF »
A Common Mechanism for Cystic Fibrosis Transmembrane Conductance Regulator Protein Activation by Genistein and Benzimidazolone Analogs.
L. Al-Nakkash, S. Hu, M. Li, and T.-C. Hwang (2001)
J. Pharmacol. Exp. Ther. 296, 464-472
   Abstract »    Full Text »
A src-like kinase activates outwardly rectifying chloride channels in CFTR-defective lymphocytes.
A. LEPPLE-WIENHUES, U. WIELAND, T. LAUN, L. HEIL, M. STERN, and F. LANG (2001)
FASEB J 15, 927-931
   Abstract »    Full Text »    PDF »
The {Delta}F508 mutation shortens the biochemical half-life of plasma membrane CFTR in polarized epithelial cells.
G. D. Heda, M. Tanwani, and C. R. Marino (2001)
Am J Physiol Cell Physiol 280, C166-C174
   Abstract »    Full Text »    PDF »
Properties of CFTR activated by the xanthine derivative X-33 in human airway Calu-3 cells.
L. Bulteau, R. Derand, Y. Mettey, T. Metaye, M. R. Morris, C. M. McNeilly, C. Folli, L. J. V. Galietta, O. Zegarra-Moran, M. M. C. Pereira, et al. (2000)
Am J Physiol Cell Physiol 279, C1925-C1937
   Abstract »    Full Text »    PDF »
Molecular heterogeneity of the Jknull phenotype: expression analysis of the Jk(S291P) mutation found in Finns.
F. Sidoux-Walter, N. Lucien, R. Nissinen, P. Sistonen, S. Henry, J. Moulds, J.-P. Cartron, and P. Bailly (2000)
Blood 96, 1566-1573
   Abstract »    Full Text »    PDF »
Activation of Protein Kinase A Modulates Trafficking of the Human Cardiac Sodium Channel in Xenopus Oocytes.
J. Zhou, J. Yi, N. Hu, A. L. George Jr, and K. T. Murray (2000)
Circ. Res. 87, 33-38
   Abstract »    Full Text »    PDF »
Heterozygous MDR3 missense mutation associated with intrahepatic cholestasis of pregnancy: evidence for a defect in protein trafficking.
P.H. Dixon, N. Weerasekera, K.J. Linton, O. Donaldson, J. Chambers, E. Egginton, J. Weaver, C. Nelson-Piercy, M. d. Swiet, G. Warnes, et al. (2000)
Hum. Mol. Genet. 9, 1209-1217
   Abstract »    Full Text »    PDF »
The Dominant Negative LQT2 Mutation A561V Reduces Wild-type HERG Expression.
A. Kagan, Z. Yu, G. I. Fishman, and T. V. McDonald (2000)
J. Biol. Chem. 275, 11241-11248
   Abstract »    Full Text »    PDF »
cAMP-dependent Cl- Channel Protein (CFTR) and Its mRNA Are Expressed in the Secretory Portion of Human Eccrine Sweat Gland.
F. Sato and K. Sato (2000)
J. Histochem. Cytochem. 48, 345-354
   Abstract »    Full Text »
Development of Substituted Benzo[c]quinolizinium Compounds as Novel Activators of the Cystic Fibrosis Chloride Channel.
F. Becq, Y. Mettey, M. A. Gray, L. J. V. Galietta, R. L. Dormer, M. Merten, T. Metaye, V. Chappe, C. Marvingt-Mounir, O. Zegarra-Moran, et al. (1999)
J. Biol. Chem. 274, 27415-27425
   Abstract »    Full Text »    PDF »
Syntaxin 1A inhibits regulated CFTR trafficking in Xenopus oocytes.
K. W. Peters, J. Qi, S. C. Watkins, and R. A. Frizzell (1999)
Am J Physiol Cell Physiol 277, C174-C180
   Abstract »    Full Text »    PDF »
Protein Kinase A Phosphorylation Alters Kvbeta 1.3 Subunit-mediated Inactivation of the Kv1.5 Potassium Channel.
Y.-G. Kwak, N. Hu, J. Wei, A. L. George Jr., T. D. Grobaski, M. M. Tamkun, and K. T. Murray (1999)
J. Biol. Chem. 274, 13928-13932
   Abstract »    Full Text »    PDF »
Cloning, characterization, and functional expression of a CNP receptor regulating CFTR in the shark rectal gland.
S. G. Aller, I. D. Lombardo, S. Bhanot, and J. N. Forrest Jr. (1999)
Am J Physiol Cell Physiol 276, C442-C449
   Abstract »    Full Text »    PDF »
Physiological Basis of Cystic Fibrosis: A Historical Perspective.
P. M. QUINTON (1999)
Physiol Rev 79, 3-22
   Abstract »    Full Text »    PDF »
Structure and Function of the CFTR Chloride Channel.
D. N. SHEPPARD and M. J. WELSH (1999)
Physiol Rev 79, 23-45
   Abstract »    Full Text »    PDF »
Control of CFTR Channel Gating by Phosphorylation and Nucleotide Hydrolysis.
D. C. GADSBY and A. C. NAIRN (1999)
Physiol Rev 79, 77-107
   Abstract »    Full Text »    PDF »
Pharmacology of CFTR Chloride Channel Activity.
B. D. SCHULTZ, A. K. SINGH, D. C. DEVOR, and R. J. BRIDGES (1999)
Physiol Rev 79, 109-144
   Abstract »    Full Text »    PDF »
Biosynthesis and Degradation of CFTR.
R. R. KOPITO (1999)
Physiol Rev 79, 167-173
   Abstract »    Full Text »    PDF »
Intracellular CFTR: Localization and Function.
N. A. BRADBURY (1999)
Physiol Rev 79, 175-191
   Abstract »    Full Text »    PDF »
A Conserved Region of the R Domain of Cystic Fibrosis Transmembrane Conductance Regulator Is Important in Processing and Function.
E. A. Pasyk, X. K. Morin, P. Zeman, E. Garami, K. Galley, L. J. Huan, Y. Wang, and C. E. Bear (1998)
J. Biol. Chem. 273, 31759-31764
   Abstract »    Full Text »    PDF »
Cystic fibrosis transmembrane conductance regulator activation by cAMP-independent mechanisms.
Z. He, S. Raman, Y. Guo, and W. W. Reenstra (1998)
Am J Physiol Cell Physiol 275, C958-C966
   Abstract »    Full Text »    PDF »
Syntaxin 1A inhibits CFTR chloride channels by means of domain-specific protein-protein interactions.
A. P. Naren, M. W. Quick, J. F. Collawn, D. J. Nelson, and K. L. Kirk (1998)
PNAS 95, 10972-10977
   Abstract »    Full Text »    PDF »
Direct Activation of Cystic Fibrosis Transmembrane Conductance Regulator Channels by 8-Cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-Diallyl-8-cyclohexylxanthine (DAX).
N. Arispe, J. Ma, K. A. Jacobson, and H. B. Pollard (1998)
J. Biol. Chem. 273, 5727-5734
   Abstract »    Full Text »    PDF »
Type II protein kinase A regulates CFTR in airway, pancreatic, and intestinal cells.
W. K. Steagall, T. J. Kelley, R. J. Marsick, and M. L. Drumm (1998)
Am J Physiol Cell Physiol 274, C819-C826
   Abstract »    Full Text »    PDF »
Effect of IBMX and alkaline phosphatase inhibitors on Cl- secretion in G551D cystic fibrosis mutant mice.
S. N. Smith, S. J. Delaney, J. R. Dorin, R. Farley, D. M. Geddes, D. J. Porteous, B. J. Wainwright, and E. W. F. W. Alton (1998)
Am J Physiol Cell Physiol 274, C492-C499
   Abstract »    Full Text »    PDF »
A functional CFTR-NBF1 is required for ROMK2-CFTR interaction.
C. M. McNicholas, M. W. Nason Jr., W. B. Guggino, E. M. Schwiebert, S. C. Hebert, G. Giebisch, and M. E. Egan (1997)
Am J Physiol Renal Physiol 273, F843-F848
   Abstract »    Full Text »    PDF »
In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant Delta F508 in murine nasal epithelium.
T. J. Kelley, K. Thomas, L. J. H. Milgram, and M. L. Drumm (1997)
PNAS 94, 2604-2608
   Abstract »    Full Text »    PDF »
Effect of Cystic Fibrosis-associated Mutations in the Fourth Intracellular Loop of Cystic Fibrosis Transmembrane Conductance Regulator.
J. F. Cotten, L. S. Ostedgaard, M. R. Carson, and M. J. Welsh (1996)
J. Biol. Chem. 271, 21279-21284
   Abstract »    Full Text »    PDF »
cAMP- and Ca2+-independent Activation of Cystic Fibrosis Transmembrane Conductance Regulator Channels by Phenylimidazothiazole Drugs.
F. Becq, B. Verrier, X.-B. Chang, J. R. Riordan, and J. W. Hanrahan (1996)
J. Biol. Chem. 271, 16171-16179
   Abstract »    Full Text »    PDF »
Alteration of the Cystic Fibrosis Transmembrane Conductance Regulator Folding Pathway.
B.-H. Qu and P. J. Thomas (1996)
J. Biol. Chem. 271, 7261-7264
   Abstract »    Full Text »    PDF »
Intracellular Loop between Transmembrane Segments IV and V of Cystic Fibrosis Transmembrane Conductance Regulator Is Involved in Regulation of Chloride Channel Conductance State.
J. Xie, M. L. Drumm, J. Ma, and P. B. Davis (1995)
J. Biol. Chem. 270, 28084-28091
   Abstract »    Full Text »    PDF »
Purification and Characterization of Recombinant Cystic Fibrosis Transmembrane Conductance Regulator from Chinese Hamster Ovary and Insect Cells.
C. R. O'Riordan, A. Erickson, C. Bear, C. Li, P. Manavalan, K. X. Wang, J. Marshall, R. K. Scheule, J. M. McPherson, S. H. Cheng, et al. (1995)
J. Biol. Chem. 270, 17033-17043
   Abstract »    Full Text »    PDF »
Mutant ([IMAGE]F508) Cystic Fibrosis Transmembrane Conductance Regulator Cl[IMAGE] Channel Is Functional When Retained in Endoplasmic Reticulum of Mammalian Cells.
E. A. Pasyk and J. K. Foskett (1995)
J. Biol. Chem. 270, 12347-12350
   Abstract »    Full Text »    PDF »
cAMP-dependent Protein Kinase-mediated Phosphorylation of Cystic Fibrosis Transmembrane Conductance Regulator Residue Ser-753 and Its Role in Channel Activation.
F. S. Seibert, J. A. Tabcharani, X.-B. Chang, A. M. Dulhanty, C. Mathews, J. W. Hanrahan, and J. R. Riordan (1995)
J. Biol. Chem. 270, 2158-2162
   Abstract »    Full Text »    PDF »
Production and characterisation of monoclonal and polyclonal antibodies to different regions of the cystic fibrosis transmembrane conductance regulator (CFTR): detection of immunologically related proteins.
J Walker, J Watson, C Holmes, A Edelman, and G Banting (1995)
J. Cell Sci. 108, 2433-2444
   Abstract »    PDF »
A multifunctional aqueous channel formed by CFTR.
H Hasegawa, W Skach, O Baker, M. Calayag, V Lingappa, and A. Verkman (1992)
Science 258, 1477-1479
   Abstract »    PDF »
Cystic fibrosis: molecular biology and therapeutic implications.
F. Collins (1992)
Science 256, 774-779
   Abstract »    PDF »
Cystic Fibrosis Mutations Lead to Carboxyl-terminal Fragments That Highlight an Early Biogenesis Step of the Cystic Fibrosis Transmembrane Conductance Regulator.
M. Van Oene, G. L. Lukacs, and J. M. Rommens (2000)
J. Biol. Chem. 275, 19577-19584
   Abstract »    Full Text »    PDF »
Conformational and Temperature-sensitive Stability Defects of the Delta F508 Cystic Fibrosis Transmembrane Conductance Regulator in Post-endoplasmic Reticulum Compartments.
M. Sharma, M. Benharouga, W. Hu, and G. L. Lukacs (2001)
J. Biol. Chem. 276, 8942-8950
   Abstract »    Full Text »    PDF »
Identification and Pharmacological Correction of a Membrane Trafficking Defect Associated with a Mutation in the Sulfonylurea Receptor Causing Familial Hyperinsulinism.
C. J. Partridge, D. J. Beech, and A. Sivaprasadarao (2001)
J. Biol. Chem. 276, 35947-35952
   Abstract »    Full Text »    PDF »
Identification of the Cystic Fibrosis Transmembrane Conductance Regulator Domains That Are Important for Interactions with ROMK2.
P. Cahill, M. W. Nason Jr., C. Ambrose, T.-Y. Yao, P. Thomas, and M. E. Egan (2000)
J. Biol. Chem. 275, 16697-16701
   Abstract »    Full Text »    PDF »


ADVERTISEMENT
Click Me!

ADVERTISEMENT
Click Me!

To Advertise     Find Products

Science. ISSN 0036-8075 (print), 1095-9203 (online)