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Cystic fibrosis transmembrane conductance regulator (CFTR) anion binding as a probe of the pore.
Mansoura MK
,
Smith SS
,
Choi AD
,
Richards NW
,
Strong TV
,
Drumm ML
,
Collins FS
,
Dawson DC
.
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We compared the effects of mutations in transmembrane segments (TMs) TM1, TM5, and TM6 on the conduction and activation properties of the cystic fibrosis transmembrane conductance regulator (CFTR) to determine which functional property was most sensitive to mutations and, thereby, to develop a criterion for measuring the importance of a particular residue or TM for anion conduction or activation. Anion substitution studies provided strong evidence for the binding of permeant anions in the pore. Anion binding was highly sensitive to point mutations in TM5 and TM6. Permeability ratios, in contrast, were relatively unaffected by the same mutations, so that anion binding emerged as the conduction property most sensitive to structural changes in CFTR. The relative insensitivity of permeability ratios to CFTR mutations was in accord with the notion that anion-water interactions are important determinants of permeability selectivity. By the criterion of anion binding, TM5 and TM6 were judged to be likely to contribute to the structure of the anion-selective pore, whereas TM1 was judged to be less important. Mutations in TM5 and TM6 also dramatically reduced the sensitivity of CFTR to activation by 3-isobutyl 1-methyl xanthine (IBMX), as expected if these TMs are intimately involved in the physical process that opens and closes the channel.
Akabas,
Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator.
1994, Pubmed,
Xenbase
Akabas,
Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator.
1994,
Pubmed
,
Xenbase
Anderson,
Demonstration that CFTR is a chloride channel by alteration of its anion selectivity.
1991,
Pubmed
Bear,
Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR).
1992,
Pubmed
Bear,
Cl- channel activity in Xenopus oocytes expressing the cystic fibrosis gene.
1991,
Pubmed
,
Xenbase
Bormann,
Mechanism of anion permeation through channels gated by glycine and gamma-aminobutyric acid in mouse cultured spinal neurones.
1987,
Pubmed
Carroll,
Alternate translation initiation codons can create functional forms of cystic fibrosis transmembrane conductance regulator.
1995,
Pubmed
,
Xenbase
Chang,
Mapping of cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion.
1994,
Pubmed
Collins,
Sticky ions in biological systems.
1995,
Pubmed
Collins,
Charge density-dependent strength of hydration and biological structure.
1997,
Pubmed
Cunningham,
cAMP-stimulated ion currents in Xenopus oocytes expressing CFTR cRNA.
1992,
Pubmed
,
Xenbase
Dani,
Lyotropic anions. Na channel gating and Ca electrode response.
1983,
Pubmed
Diamond,
A cross-species analysis of the cystic fibrosis transmembrane conductance regulator. Potential functional domains and regulatory sites.
1991,
Pubmed
Drumm,
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
1991,
Pubmed
,
Xenbase
Epstein,
Active transport of chloride by the teleost gill: inhibition by thiocyanate.
1973,
Pubmed
Golla,
Identification of a novel missense mutation (G314E) in exon 7 of the cystic fibrosis transmembrane conductance regulator gene identified in a CF patient with pancreatic sufficiency.
1994,
Pubmed
Guillermit,
A novel mutation in exon 3 of the CFTR gene.
1993,
Pubmed
HARRIS,
Anion interaction in frog muscle.
1958,
Pubmed
HUTTER,
Effect of nitrate and other anions on the membrane resistance of frog skeletal muscle.
1959,
Pubmed
Hagiwara,
Mechanism of anion permeation through the muscle fibre membrane of an elasmobranch fish, Taeniura lymma.
1974,
Pubmed
Halm,
Anion permeation in an apical membrane chloride channel of a secretory epithelial cell.
1992,
Pubmed
Hipper,
Mutations in the putative pore-forming domain of CFTR do not change anion selectivity of the cAMP activated Cl- conductance.
1995,
Pubmed
,
Xenbase
Holmgren,
On the use of thiol-modifying agents to determine channel topology.
1996,
Pubmed
,
Xenbase
Jan,
Potassium channels and their evolving gates.
1994,
Pubmed
Kirsch,
A single nonpolar residue in the deep pore of related K+ channels acts as a K+:Rb+ conductance switch.
1992,
Pubmed
Linsdell,
Flickery block of single CFTR chloride channels by intracellular anions and osmolytes.
1996,
Pubmed
Linsdell,
Permeability of wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride channels to polyatomic anions.
1997,
Pubmed
Linsdell,
Disulphonic stilbene block of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in a mammalian cell line and its regulation by a critical pore residue.
1996,
Pubmed
Marshall,
Identification and localization of a dogfish homolog of human cystic fibrosis transmembrane conductance regulator.
1991,
Pubmed
McCarty,
Voltage-dependent block of the cystic fibrosis transmembrane conductance regulator Cl- channel by two closely related arylaminobenzoates.
1993,
Pubmed
,
Xenbase
McDonough,
Novel pore-lining residues in CFTR that govern permeation and open-channel block.
1994,
Pubmed
Nasr,
Novel missense mutation (G314R) in a cystic fibrosis patient with hepatic failure.
1996,
Pubmed
,
Xenbase
Norne,
Anion binding properties of human serum albumin from halide ion quadrupole relaxation.
1975,
Pubmed
Norne,
Pt(CN)2-4 and Au(CN)-2: potential general probes for anion-binding sites of proteins. 35Cl and 81Br nuclear-magnetic-resonance studies.
1975,
Pubmed
Overholt,
On the mechanism of rectification of the isoproterenol-activated chloride current in guinea-pig ventricular myocytes.
1993,
Pubmed
Overholt,
Rectification of whole cell cystic fibrosis transmembrane conductance regulator chloride current.
1995,
Pubmed
Pande,
Thiocyanate binding with modified bovine plasma albumins.
1970,
Pubmed
Record,
Thermodynamic analysis of ion effects on the binding and conformational equilibria of proteins and nucleic acids: the roles of ion association or release, screening, and ion effects on water activity.
1978,
Pubmed
Riordan,
Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.
1989,
Pubmed
Sheppard,
Effect of ATP-sensitive K+ channel regulators on cystic fibrosis transmembrane conductance regulator chloride currents.
1992,
Pubmed
Sheppard,
Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties.
1993,
Pubmed
Smit,
Functional roles of the nucleotide-binding folds in the activation of the cystic fibrosis transmembrane conductance regulator.
1993,
Pubmed
,
Xenbase
Strong,
Cystic fibrosis gene mutation in two sisters with mild disease and normal sweat electrolyte levels.
1991,
Pubmed
Tabcharani,
Halide permeation in wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride channels.
1997,
Pubmed
Tabcharani,
Multi-ion pore behaviour in the CFTR chloride channel.
1993,
Pubmed
Tabcharani,
The cystic fibrosis transmembrane conductance regulator chloride channel. Iodide block and permeation.
1992,
Pubmed
Taglialatela,
Regulation of K+/Rb+ selectivity and internal TEA blockade by mutations at a single site in K+ pores.
1993,
Pubmed
Takeuchi,
Anion interaction at the inhibitory post-synaptic membrane of the crayfish neuromuscular junction.
1971,
Pubmed
Tata,
Cloning the mouse homolog of the human cystic fibrosis transmembrane conductance regulator gene.
1991,
Pubmed
Tucker,
Identification and developmental expression of the Xenopus laevis cystic fibrosis transmembrane conductance regulator gene.
1992,
Pubmed
,
Xenbase
Vaughan,
Chloride-thiocyanate interactions in frog muscle anion-conducting channels at pH 5.
1987,
Pubmed
,
Xenbase
White,
Probes of the conduction process of a voltage-gated Cl- channel from Torpedo electroplax.
1981,
Pubmed
Wilkinson,
CFTR: the nucleotide binding folds regulate the accessibility and stability of the activated state.
1996,
Pubmed
,
Xenbase
Wright,
Anion selectivity in biological systems.
1977,
Pubmed
Yang,
Molecular basis of charge movement in voltage-gated sodium channels.
1996,
Pubmed
Yim,
Polymer membrane-based ion-, gas- and bio-selective potentiometric sensors.
1993,
Pubmed
