Development of CFTR Structure

Keywords

Ion channel, Medicine, Pharmacology, Clinical pharmacology, Chemistry

References

• Structural cues involved in endoplasmic reticulum degradation of G85E and G91R mutant cystic fibrosis transmembrane conductance regulator.Flavie Bondois — 1997
• ATP binding to the motor domain from an ABC transporter drives formation of a nucleotide sandwich dimer.Philip C. Smith, Nathan K. Karpowich, Linda Millen — 2002
• Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP.Gergely L. Lukács, Asmaa Mohamed, Norbert Kartner — 1994
• Overview: ABC Transporters and Human DiseaseMichael M. Gottesman, Suresh V. Ambudkar — 2001
• ABC proteins : from bacteria to manI.B. Holland, Susan P.C. Cole, Karl Kuchler — 2003
• The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide.Philip Thomas, P Shenbagamurthi, John Sondek — 1992
• The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradationGeoffrey C. Meacham, Cam Patterson, Wenyue Zhang — 2000
• Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator.S. Pind, J.R. Riordan, Donald B. Williams — 1994
• The Escherichia coli ATP‐binding cassette (ABC) proteinsKenneth J. Linton, Christopher F. Higgins — 1998
• Expression and intracellular processing of chimeric and mutant CFTR molecules.Jean-François Pollet, J. van Geffel, Evelyn Stevens — 2000
• Mechanism of chloride permeation in the cystic fibrosis transmembrane conductance regulator chloride channelPaul Linsdell — 2006
• Alteration of the Cystic Fibrosis Transmembrane Conductance Regulator Folding PathwayBao-He Qu, Philip Thomas — 1996
• The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells.Gergely L. Lukács, X.B. Chang, Christine E. Bear — 1993
• The human ATP-binding cassette (ABC) transporter superfamily.Michael Dean, Yannick Hamon, Giovanna Chimini — 2001
• Molecular models of the open and closed states of the whole human CFTR proteinJean‐Paul Mornon, Pierre Lehn, Isabelle Callebaut — 2009
• CFTR regulatory region interacts with NBD1 predominantly via multiple transient helicesJennifer M. Baker, Rhea P. Hudson, Voula Kanelis — 2007
• Mapping of Interdomain Interfaces Required for the Functional Architecture of Yor1p, a Eukaryotic ATP-binding Cassette (ABC) Transporter*Silvère Pagant, Ethan Y. Brovman, John J. Halliday — 2008
• CFTR (ABCC7) is a hydrolyzable-ligand-gated channelAndrei A. Aleksandrov, Luba A. Aleksandrov, John R. Riordan — 2007
• Cooperative, ATP-dependent Association of the Nucleotide Binding Cassettes during the Catalytic Cycle of ATP-binding Cassette Transporters*Jonathan Moody, Linda Millen, Derk D. Binns — 2002
• Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis.Seng H. Cheng, Richard J. Gregory, John Marshall — 1990

Cited by

• Involvement of the heterodimeric interface region of the nucleotide binding domain-2 (NBD2) in the CFTR quaternary structure and membrane stability.Julien Micoud, Sylvain Chauvet, K.E. Ludwig Scheckenbach — 2015
• Repairing mutated proteins – development of small molecules targeting defects in the cystic fibrosis transmembrane conductance regulatorDaniel Merk, Manfred Schubert‐Zsilavecz — 2013
• Functional architecture of the CFTR chloride channelPaul Linsdell — 2014
• Molecular modelling approaches for cystic fibrosis transmembrane conductance regulator studies.Norbert Odolczyk, Piotr Zielenkiewicz — 2014
• Novel pharmacological strategies to treat cystic fibrosis.John W. Hanrahan, Heidi M. Sampson, David Y. Thomas — 2013
• Discovery heralds new approach to the treatment of cystic fibrosis.John Lowe — 2014
• Cystic fibrosis transmembrane conductance regulator chloride channel blockers: Pharmacological, biophysical and physiological relevance.Paul Linsdell — 2014
• MRP1 and its role in anticancer drug resistanceJamie F. Lu, Deep Pokharel, Mary Bebawy — 2015
• A single amino acid substitution in CFTR converts ATP to an inhibitory ligandWen‐Ying Lin, Kang‐Yang Jih, Tzyh‐Chang Hwang — 2014
• Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation*Xiaoyan Gong, Annette Ahner, Ariel Roldán — 2015
• Formulation and efficacy of liposome-encapsulated azithromycin for pulmonary infection due to Pseudomonias AeruginosaVenkata Saran Tejaswi Solleti — 2016
• Chaperonin-based biolayer interferometry to assess the kinetic stability of metastable, aggregation-prone proteinsWendy Lea, Pierce T. O’Neil, Alexandra J. Machen — 2016
• Gene delivery to the lungs: pulmonary gene therapy for cystic fibrosisIlia Villate-Beitia, Jon Zárate Sesma, Gustavo Puras — 2017
• Structure-Function Relationships of CFTR in Health and Disease: The Pancreas StoryMohamad Bouhamdan — 2018
• Physiological study of pulmonary involvement in adults with cystic fibrosis through simulated modeling of different clinical scenariosAntonio Alberto Rodríguez Sousa, Jesús M. Barandica, Jonathan P. Miller — 2018
• Mechanisms of ANO1 channel activation in sensory neuronsShihab Shah — 2017
• Modulation of Proteostasis for the Efficient Intracellular Transport of the ΔF508 Mutant of Cystic Fibrosis Transmembrane Conductance RegulatorR. Hegde — 2014
• Co-translational folding of the first transmembrane domain of ABC-transporter CFTR is supported by assembly with the first cytosolic domainBertrand Kleizen, Marcel van Willigen, Marjolein Mijnders — 2020
• Role of transmembrane spanning domain 1 in cystic fibrosis transmembrane conductance regulator foldingAnna E. Patrick, Linda Millen, Philip Thomas — 2021
• Molecular Mechanisms In Cftr-F508del Degradation And The Functional Defect Of Cftr Absence In RabbitsCarthic Rajagopalan — 2019

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