CFTR (ABCC7) is a hydrolyzable-ligand-gated channel

No abstract is available for this paper.

• Publication year

  2007

• Venue

  Pflügers Archiv: European Journal of Physiology

• Publication date

  2007-02-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1007/s00424-006-0140-zPMID 17021796
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• The ABC protein turned chloride channel whose failure causes cystic fibrosis

  2006cited by this paper
• Nucleotide-binding Domains of Cystic Fibrosis Transmembrane Conductance Regulator, an ABC Transporter, Catalyze Adenylate Kinase Activity but Not ATP Hydrolysis*

  2006cited by this paper
• Assembly of functional CFTR chloride channels.

  2005cited by this paper
• ADP inhibits function of the ABC transporter cystic fibrosis transmembrane conductance regulator via its adenylate kinase activity.

  2005cited by this paper
• CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains

  2005influential reference
• Preferential Phosphorylation of R-domain Serine 768 Dampens Activation of CFTR Channels by PKA

  2005cited by this paper
• Impact of the ΔF508 Mutation in First Nucleotide-binding Domain of Human Cystic Fibrosis Transmembrane Conductance Regulator on Domain Folding and Structure*

  2005cited by this paper
• Purification and Crystallization of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)*

  2004cited by this paper
• Structure of nucleotide‐binding domain 1 of the cystic fibrosis transmembrane conductance regulator

  2004cited by this paper
• Protein kinase‐independent activation of CFTR by phosphatidylinositol phosphates

  2004cited by this paper
• Dibasic phosphorylation sites in the R domain of CFTR have stimulatory and inhibitory effects on channel activation.

  2004cited by this paper
• ABC proteins : from bacteria to man

  2003cited by this paper
• Phosphorylation of protein kinase C sites in NBD1 and the R domain control CFTR channel activation by PKA

  2003influential reference
• Nucleotide dependent monomer/dimer equilibrium of OpuAA, the nucleotide-binding protein of the osmotically regulated ABC transporter OpuA from Bacillus subtilis.

  2003cited by this paper
• Stimulatory and inhibitory protein kinase C consensus sequences regulate the cystic fibrosis transmembrane conductance regulator

  2003cited by this paper
• On the Mechanism of MgATP-dependent Gating of CFTR Cl− Channels

  2003cited by this paper
• Prolonged Nonhydrolytic Interaction of Nucleotide with CFTR's NH2-terminal Nucleotide Binding Domain and its Role in Channel Gating

  2003cited by this paper
• Control of dynamic CFTR selectivity by glutamate and ATP in epithelial cells

  2003cited by this paper
• Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epithelia

  2003cited by this paper
• CHAPTER 29 – THE CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR (ABCC7)

  2003cited by this paper
• Nucleoside triphosphate pentose ring impact on CFTR gating and hydrolysis

  2002cited by this paper
• Cyclic nucleotide-gated ion channels.

  2002cited by this paper
• A Maxwell's Demon type of membrane transport: possibility for active transport by ABC-type transporters?

  2002cited by this paper
• ATP binding to the motor domain from an ABC transporter drives formation of a nucleotide sandwich dimer.

  2002cited by this paper
• Cooperative, ATP-dependent Association of the Nucleotide Binding Cassettes during the Catalytic Cycle of ATP-binding Cassette Transporters*

  2002cited by this paper
• The First Nucleotide Binding Domain of Cystic Fibrosis Transmembrane Conductance Regulator Is a Site of Stable Nucleotide Interaction, whereas the Second Is a Site of Rapid Turnover*

  2002cited by this paper
• cAMP-independent phosphorylation activation of CFTR by G proteins in native human sweat duct.

  2001cited by this paper
• A conditional probability analysis of cystic fibrosis transmembrane conductance regulator gating indicates that ATP has multiple effects during the gating cycle

  2001cited by this paper
• Ionic channels of excitable membranes

  2001cited by this paper
• Pkc-Mediated Stimulation of Amphibian Cftr Depends on a Single Phosphorylation Consensus Site. Insertion of This Site Confers Pkc Sensitivity to Human Cftr

  2001cited by this paper
• Polarized Signaling via Purinoceptors in Normal and Cystic Fibrosis Airway Epithelia

  2001cited by this paper
• Apical Heterotrimeric G-proteins Activate CFTR in the Native Sweat Duct

  2001cited by this paper
• Compartmentalized autocrine signaling to cystic fibrosis transmembrane conductance regulator at the apical membrane of airway epithelial cells

  2001cited by this paper
• Regulation of the Cystic Fibrosis Transmembrane Conductance Regulator Cl− Channel by Its R Domain*

  2001influential reference
• Differential Interactions of Nucleotides at the Two Nucleotide Binding Domains of the Cystic Fibrosis Transmembrane Conductance Regulator*

  2001influential reference
• Severed Channels Probe Regulation of Gating of Cystic Fibrosis Transmembrane Conductance Regulator by Its Cytoplasmic Domains

  2000cited by this paper
• The non‐hydrolytic pathway of cystic fibrosis transmembrane conductance regulator ion channel gating

  2000cited by this paper
• ATP hydrolysis by a CFTR domain: pharmacology and effects of G551D mutation.

  2000cited by this paper
• Nucleotide-binding domain 1 of cystic fibrosis transmembrane conductance regulator production of a suitable protein for structural studies.

  2000cited by this paper
• Regulation of CFTR Cl- channel gating by ATP binding and hydrolysis.

  2000cited by this paper
• Gating of Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channels by Adenosine Triphosphate Hydrolysis

  1999cited by this paper
• Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis.

  1999influential reference
• Biosynthesis and degradation of CFTR.

  1999cited by this paper
• Structure and function of the CFTR chloride channel.

  1999cited by this paper
• Nucleotide Occlusion in the Human Cystic Fibrosis Transmembrane Conductance Regulator

  1999cited by this paper
• Walker mutations reveal loose relationship between catalytic and channel-gating activities of purified CFTR (cystic fibrosis transmembrane conductance regulator).

  1999influential reference
• Influence of phosphorylation by protein kinase A on CFTR at the cell surface and endoplasmic reticulum.

  1999cited by this paper
• Energy, Life, and ATP

  1998cited by this paper
• Regulation of CFTR ion channel gating by MgATP

  1998influential reference
• The CFTR Chloride Channel: Nucleotide Interactions and Temperature-dependent Gating

  1998cited by this paper
• Energy, Life, and ATP (Nobel Lecture).

  1998cited by this paper
• A recombinant polypeptide model of the second nucleotide‐binding fold of the cystic fibrosis transmembrane conductance regulator functions as an active ATPase, GTPase and adenylate kinase

  1997cited by this paper
• CFTR activation: additive effects of stimulatory and inhibitory phosphorylation sites in the R domain.

  1997cited by this paper
• ATP hydrolysis cycles and mechanism in P-glycoprotein and CFTR.

  1997cited by this paper
• Block by MOPS reveals a conformation change in the CFTR pore produced by ATP hydrolysis.

  1997cited by this paper
• A recombinant polypeptide model of the second predicted nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator is a GTP‐binding protein

  1996influential reference
• Lack of Conventional ATPase Properties in CFTR Chloride Channel Gating

  1996cited by this paper
• Mutation of R555 in CFTR-delta F508 enhances function and partially corrects defective processing.

  1996cited by this paper
• ATPase Activity of the Cystic Fibrosis Transmembrane Conductance Regulator*

  1996cited by this paper
• Expression and functional properties of the second predicted nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator fused to glutathione-S-transferase.

  1995cited by this paper
• Conformational states of CFTR associated with channel gating: the role ATP binding and hydrolysis.

  1995influential reference
• Degradation of CFTR by the ubiquitin-proteasome pathway.

  1995cited by this paper
• The First Nucleotide Binding Fold of the Cystic Fibrosis Transmembrane Conductance Regulator Can Function as an Active ATPase (*)

  1995cited by this paper
• Regulation of CFTR Cl- channel gating by ADP and ATP analogues

  1995cited by this paper
• Expression and functional properties of the second predicted nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator fused to

  1995cited by this paper
• The Two Nucleotide-binding Domains of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Have Distinct Functions in Controlling Channel Activity (*)

  1995cited by this paper
• Phosphorylation by cAMP-dependent protein kinase causes a conformational change in the R domain of the cystic fibrosis transmembrane conductance regulator.

  1994cited by this paper
• Coupling of CFTR Cl− channel gating to an ATP hydrolysis cycle

  1994cited by this paper
• Regulation of CFTR channel gating.

  1994cited by this paper
• Regulation of the gating of cystic fibrosis transmembrane conductance regulator C1 channels by phosphorylation and ATP hydrolysis.

  1994cited by this paper
• ATP alters current fluctuations of cystic fibrosis transmembrane conductance regulator: evidence for a three-state activation mechanism

  1994cited by this paper
• Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by negative charge in the R domain.

  1993cited by this paper
• The cystic fibrosis transmembrane conductance regulator.

  1993influential reference
• Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites.

  1993cited by this paper
• Interaction of nucleotides with membrane-associated cystic fibrosis transmembrane conductance regulator.

  1993cited by this paper
• Control of CFTR chloride conductance by ATP levels through non-hydrolytic binding

  1992cited by this paper
• Nucleoside triphosphates are required to open the CFTR chloride channel.

  1991cited by this paper
• Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel.

  1991cited by this paper
• Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis.

  1990cited by this paper
• Identification of the cystic fibrosis gene: chromosome walking and jumping.

  1989cited by this paper
• Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.

  1989cited by this paper
• Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA

  1989cited by this paper
• Identification of the cystic fibrosis gene: Chromosome walking and jumping

  1989cited by this paper
• Cl- channels in CF: lack of activation by protein kinase C and cAMP-dependent protein kinase.

  1989influential reference
• Phosphorylation fails to activate chloride channels from cystic fibrosis airway cells

  1987cited by this paper
• Abnormal ion permeation through cystic fibrosis respiratory epithelium.

  1983cited by this paper
• Chloride impermeability in cystic fibrosis

  1983cited by this paper
• ON THE NATURE OF ALLOSTERIC TRANSITIONS: A PLAUSIBLE MODEL.

  1965cited by this paper

• The ATP‐bound inward‐open conformation of ABCC4 reveals asymmetric ATP binding for substrate transport

  2024cites this paper
• A Proximity Complementation Assay to Identify Small Molecules That Enhance the Traffic of ABCA4 Misfolding Variants

  2024cites this paper
• The interactome of cystic fibrosis transmembrane conductance regulator and its role in male fertility: A critical review

  2024cites this paper
• A conserved WXXE motif is an apical delivery determinant of ABC transporter C subfamily isoforms

  2023cites this paper
• Membrane Lipids and Transporter Function.

  2021cites this paper
• CFTR Lifecycle Map—A Systems Medicine Model of CFTR Maturation to Predict Possible Active Compound Combinations

  2021cites this paper
• Varied clinical significance of ATP-binding cassette C sub-family members for lung adenocarcinoma

  2021cites this paper
• David Christopher Gadsby. 26 March 1947—9 March 2019

  2020cites this paper
• Focal adhesion kinase and osmotic responses in ionocytes of Fundulus heteroclitus, a euryhaline teleost fish.

  2019cites this paper
• Cholesterol Interaction Directly Enhances Intrinsic Activity of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

  2019cites this paper
• Lipid interactions enhance activation and potentiation of cystic fibrosis transmembrane conductance regulator (CFTR)

  2018cites this paper
• The role of intracellular Ca2+ in cigarette smoke-induced CFTR internalisation

  2017cites this paper
• Genome-wide identification of ATP-binding cassette (ABC) transporters and their roles in response to polycyclic aromatic hydrocarbons (PAHs) in the copepod Paracyclopina nana.

  2017cites this paper
• Predicting liver toxicity on basis of transporter interaction profiles

  2016cites this paper
• ThemeGating of CFTR on an Equilibrium

  2016cites this paper
• Current insights into the role of PKA phosphorylation in CFTR channel activity and the pharmacological rescue of cystic fibrosis disease-causing mutants

  2016cites this paper
• Mechanosensitive activation of CFTR by increased cell volume and hydrostatic pressure but not shear stress.

  2015cites this paper
• The Concise Guide to PHARMACOLOGY 2015/16: Other ion channels

  2015cites this paper
• Mutating the Conserved Q-loop Glutamine 1291 Selectively Disrupts Adenylate Kinase-dependent Channel Gating of the ATP-binding Cassette (ABC) Adenylate Kinase Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) and Reduces Channel Function in Primary Human Airway Epithelia*

  2015cites this paper
• Rescuing Trafficking Mutants of the ATP-binding Cassette Protein, ABCA4, with Small Molecule Correctors as a Treatment for Stargardt Eye Disease*

  2015cites this paper
• Characterization of ATP-binding Cassette Transporter Genes in Silkworm, Bombyx Mori

  2015cites this paper
• Structural diversity of ABC transporters

  2014cites this paper
• An Electrostatic Interaction at the Tetrahelix Bundle Promotes Phosphorylation-dependent Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channel Opening*

  2014cites this paper
• Conformational dynamics of the nucleotide binding domains and the power stroke of a heterodimeric ABC transporter

  2014cites this paper
• CFTR structure and cystic fibrosis.

  2014cites this paper
• Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein Expressed in Saccharomyces cerevisiae

  2014cites this paper
• 2,4,7-Triphenylbenzimidazole: the monomeric unit of supramolecular helical rod-like transmembrane transporters

  2014cites this paper
• Conserved Allosteric Hot Spots in the Transmembrane Domains of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channels and Multidrug Resistance Protein (MRP) Pumps*

  2014cites this paper
• Current advances on ABC drug transporters in fish.

  2014cites this paper
• Expression and Purification of the Cystic Fibrosis Transmembrane Conductance Regulator from Saccharomyces cerevisiae for High-Resolution Structural Studies

  2014cites this paper
• Tissue-Specific Transcript Profiling for ABC Transporters in the Sequestering Larvae of the Phytophagous Leaf Beetle Chrysomela populi

  2014cites this paper
• ATP and AMP Mutually Influence Their Interaction with the ATP-binding Cassette (ABC) Adenylate Kinase Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) at Separate Binding Sites*

  2013cites this paper
• Diverting the downhill flow of auxin to steer growth during tropisms.

  2013cites this paper
• Cystic fibrosis transmembrane conductance regulator (ABCC7) structure.

  2013cites this paper
• The Concise Guide to Pharmacology 2013/14: Ion Channels

  2013cites this paper
• Converting Nonhydrolyzable Nucleotides to Strong Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Agonists by Gain of Function (GOF) Mutations*

  2013cites this paper
• Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle

  2013cites this paper
• Cystic Fibrosis Transmembrane Conductance Regulator ( ABCC 7 ) Structure

  2013cites this paper
• Nonequilibrium gating of CFTR on an equilibrium theme.

  2012cites this paper
• Cystic fibrosis transmembrane conductance regulator in the gills of the climbing perch, Anabas testudineus, is involved in both hypoosmotic regulation during seawater acclimation and active ammonia excretion during ammonia exposure

  2012cites this paper
• Demonstration of Phosphoryl Group Transfer Indicates That the ATP-binding Cassette (ABC) Transporter Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Exhibits Adenylate Kinase Activity*

  2012cites this paper
• Development of CFTR Structure

  2012cites this paper
• CFTR: An ion channel with a transporter-type energy-coupling mechanism

  2012cites this paper
• Hematopoietic Stem/Progenitor Cells Express Functional Mitochondrial Energy-Dependent Cystic Fibrosis Transmembrane Conductance Regulator

  2012cites this paper
• Dancing with the sterols: critical roles for ABCG1, ABCA1, miRNAs, and nuclear and cell surface receptors in controlling cellular sterol homeostasis.

  2012cites this paper
• Multidrug resistance-associated ABC transporters – too much of one thing, good for nothing

  2012cites this paper
• La protéine CFTR : Implication et cible thérapeutique dans la maladie osseuse chez les patients atteints de mucoviscidose.

  2012cites this paper
• Guide to Receptors and Channels (GRAC), 5th edition

  2011cites this paper
• ENZYMES

  2011cites this paper
• Regulation of ABC transporter function via phosphorylation by protein kinases.

  2011cites this paper
• Understanding protein kinase CK2 mis-regulation upon F508del CFTR expression

  2011cites this paper
• A Unified View of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gating: Combining the Allosterism of a Ligand-gated Channel with the Enzymatic Activity of an ATP-binding Cassette (ABC) Transporter*

  2011cites this paper
• Contribution à l’amélioration des connaissances sur la relation génotype-phénotype dans la mucoviscidose et caractérisation phénotypique de l’inflammation pulmonaire

  2011cites this paper
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  2011cites this paper
• CATALYTIC RECEPTORS

  2011cites this paper
• TRANSPORTERS

  2011cites this paper
• Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment.

  2011cites this paper
• ATP hydrolysis-dependent asymmetry of the conformation of CFTR channel pore

  2011cites this paper
• Consultants

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• The H-loop in the Second Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator is Required for Efficient Chloride Channel Closing

  2010cites this paper
• In silico modelling of transporter-mediated xenobiotic flux through cells

  2010cites this paper
• Understanding nucleotide binding and CFTR ion channel gating: how many cycles?

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• CFTR: Break a pump, make a channel

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• Regulation of hepatic ABCC transporters by xenobiotics and in disease states

  2010cites this paper
• Inwardly rectifying potassium channels: their structure, function, and physiological roles.

  2010cites this paper
• Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR.

  2010cites this paper
• C Terminus of Nucleotide Binding Domain 1 Contains Critical Features for Cystic Fibrosis Transmembrane Conductance Regulator Trafficking and Activation*

  2010cites this paper
• Estudio de la expresión del gen CISD1 mediada por la actividad del CFTR

  2010cites this paper
• Mechanistic Insight into Control of CFTR by AMPK*S⃞

  2009cites this paper
• Gating of the CFTR Cl− channel by ATP‐driven nucleotide‐binding domain dimerisation

  2009cites this paper
• Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator

  2009cites this paper
• Guide to Receptors and Channels (GRAC), 4th edition

  2009cites this paper
• Assessment of the CFTR and ENaC association.

  2009cites this paper
• Curcumin Cross-links Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Polypeptides and Potentiates CFTR Channel Activity by Distinct Mechanisms*

  2009cites this paper
• ION CHANNELS

  2009cites this paper
• CFTR Cl– channel functional regulation by phosphorylation of focal adhesion kinase at tyrosine 407 in osmosensitive ion transporting mitochondria rich cells of euryhaline killifish

  2009influential citation
• Application of rate-equilibrium free energy relationship analysis to nonequilibrium ion channel gating mechanisms

  2009cites this paper
• A Small-Molecule Modulator Interacts Directly with ΔPhe508-CFTR to Modify Its ATPase Activity and Conformational Stability

  2009cites this paper
• The ABC transporter gene family of Daphnia pulex

  2009cites this paper
• The intact CFTR protein mediates ATPase rather than adenylate kinase activity.

  2008cites this paper
• State-dependent Access of Anions to the Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Pore*

  2008cites this paper
• CFTR function and prospects for therapy.

  2008cites this paper
• 49th Annual Meeting Deutsche Gesellschaft für Experimentelle und Klinische Pharmakologie und Toxikologie, Mainz, March 11-13, 2008

  2008cites this paper
• Interplay of conjugating enzymes with OATP uptake transporters and ABCC/MRP efflux pumps in the elimination of drugs

  2008cites this paper
• Imaging CFTR in its native environment

  2008cites this paper
• Molecular basis for the ATPase activity of CFTR.

  2008cites this paper
• Multiple Membrane-Cytoplasmic Domain Contacts in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mediate Regulation of Channel Gating*

  2008cites this paper
• Chemotherapy-induced resistance by ATP-binding cassette transporter genes.

  2007cites this paper
• Role of CFTR’s intrinsic adenylate kinase activity in gating of the Cl− channel

  2007cites this paper
• Molecular Proximity of Cystic Fibrosis Transmembrane Conductance Regulator and Epithelial Sodium Channel Assessed by Fluorescence Resonance Energy Transfer*

  2007cites this paper
• University of Groningen Structural diversity of ABC transporters

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