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Proc Natl Acad Sci U S A
1998 Mar 03;955:2674-9. doi: 10.1073/pnas.95.5.2674.
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Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator.
Schwiebert EM, Morales MM, Devidas S, Egan ME, Guggino WB.
???displayArticle.abstract??? CFTR is a cyclic AMP (cAMP)-activated chloride (Cl-) channel and a regulator of outwardly rectifying Cl- channels (ORCCs) in airway epithelia. CFTR regulates ORCCs by facilitating the release of ATP out of cells. Once released from cells, ATP stimulates ORCCs by means of a purinergic receptor. To define the domains of CFTR important for Cl- channel function and/or ORCC regulator function, mutant CFTRs with N- and C-terminal truncations and selected individual amino acid substitutions were created and studied by transfection into a line of human airway epithelial cells from a cystic fibrosis patient (IB3-1) or by injection of in vitro transcribed complementary RNAs (cRNAs) into Xenopus oocytes. Two-electrode voltage clamp recordings, 36Cl- efflux assays, and whole cell patch-clamp recordings were used to assay for the Cl- channel function of CFTR and for its ability to regulate ORCCs. The data showed that the first transmembrane domain (TMD-1) of CFTR, especially predicted alpha-helices 5 and 6, forms an essential part of the Cl- channel pore, whereas the first nucleotide-binding and regulatory domains (NBD1/R domain) are essential for its ability to regulate ORCCs. Finally, the data show that the ability of CFTR to function as a Cl- channel and a conductance regulator are not mutually exclusive; one function could be eliminated while the other was preserved.
Abraham,
Cystic fibrosis transmembrane conductance regulator and adenosine triphosphate.
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Cloning of the beta cell high-affinity sulfonylurea receptor: a regulator of insulin secretion.
1995,
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Promiscuous coupling between the sulphonylurea receptor and inwardly rectifying potassium channels.
1996,
Pubmed
,
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Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation.
1986,
Pubmed Carroll,
Alternate translation initiation codons can create functional forms of cystic fibrosis transmembrane conductance regulator.
1995,
Pubmed
,
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Defective regulation of outwardly rectifying Cl- channels by protein kinase A corrected by insertion of CFTR.
1992,
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Two cystic fibrosis transmembrane conductance regulator mutations have different effects on both pulmonary phenotype and regulation of outwardly rectified chloride currents.
1995,
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,
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1993,
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1992,
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The ABC of channel regulation.
1995,
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Reconstitution of IKATP: an inward rectifier subunit plus the sulfonylurea receptor.
1995,
Pubmed Inagaki,
A family of sulfonylurea receptors determines the pharmacological properties of ATP-sensitive K+ channels.
1996,
Pubmed Ismailov,
Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator.
1996,
Pubmed Isomoto,
A novel sulfonylurea receptor forms with BIR (Kir6.2) a smooth muscle type ATP-sensitive K+ channel.
1996,
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Interaction between cystic fibrosis transmembrane conductance regulator and outwardly rectified chloride channels.
1995,
Pubmed Kunzelmann,
Inhibition of epithelial Na+ currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator.
1997,
Pubmed
,
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Sensitivity of a renal K+ channel (ROMK2) to the inhibitory sulfonylurea compound glibenclamide is enhanced by coexpression with the ATP-binding cassette transporter cystic fibrosis transmembrane regulator.
1996,
Pubmed
,
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Both the wild type and a functional isoform of CFTR are expressed in kidney.
1996,
Pubmed
,
Xenbase Philipson,
Pas de deux or more: the sulfonylurea receptor and K+ channels.
1995,
Pubmed Reddy,
Failure of the cystic fibrosis transmembrane conductance regulator to conduct ATP.
1996,
Pubmed Reisin,
The cystic fibrosis transmembrane conductance regulator is a dual ATP and chloride channel.
1994,
Pubmed Riordan,
Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.
1989,
Pubmed Schwiebert,
CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP.
1995,
Pubmed Schwiebert,
Both CFTR and outwardly rectifying chloride channels contribute to cAMP-stimulated whole cell chloride currents.
1994,
Pubmed Sheppard,
Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties.
1993,
Pubmed Stutts,
CFTR as a cAMP-dependent regulator of sodium channels.
1995,
Pubmed Tabcharani,
Multi-ion pore behaviour in the CFTR chloride channel.
1993,
Pubmed Valverde,
Volume-regulated chloride channels associated with the human multidrug-resistance P-glycoprotein.
1992,
Pubmed Valverde,
The multidrug resistance P-glycoprotein modulates cell regulatory volume decrease.
1996,
Pubmed Zeitlin,
A cystic fibrosis bronchial epithelial cell line: immortalization by adeno-12-SV40 infection.
1991,
Pubmed
