Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy

CLC channels and transporters: Proteins with borderline personalities

(2010) Alessio Accardi et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

An Iris-Like Mechanism of Pore Dilation in the CorA Magnesium Transport System

(2010) Nilmadhab Chakrabarti et al.   BIOPHYSICAL JOURNAL

Regulation of conductance by the number of fixed positive charges in the intracellular vestibule of the CFTR chloride channel pore

(2010) Jing-Jun Zhou et al.   JOURNAL OF GENERAL PHYSIOLOGY

A Chemical Corrector Modifies the Channel Function of F508del-CFTR

(2010) P. K. Chiaw et al.   MOLECULAR PHARMACOLOGY

Correction of the  Phe508 Cystic Fibrosis Transmembrane Conductance Regulator Trafficking Defect by the Bioavailable Compound Glafenine

(2010) R. Robert et al.   MOLECULAR PHARMACOLOGY

ATP-independent CFTR channel gating and allosteric modulation by phosphorylation

(2010) Wei Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant

(2010) Shane Atwell et al.   PROTEIN ENGINEERING DESIGN & SELECTION

Cystic Fibrosis Transmembrane Conductance Regulator: Using Differential Reactivity toward Channel-Permeant and Channel-Impermeant Thiol-Reactive Probes To Test a Molecular Model for the Pore

(2009) Christopher Alexander et al.   BIOCHEMISTRY

Molecular models of the open and closed states of the whole human CFTR protein

(2009) Jean-Paul Mornon et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Functional Rescue of DeltaF508-CFTR by Peptides Designed to Mimic Sorting Motifs

(2009) Patrick Kim Chiaw et al.   CHEMISTRY & BIOLOGY

Gating of the CFTR Cl−channel by ATP-driven nucleotide-binding domain dimerisation

(2009) Tzyh-Chang Hwang et al.   JOURNAL OF PHYSIOLOGY-LONDON

Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator

(2009) Andrei A. Aleksandrov et al.   JOURNAL OF PHYSIOLOGY-LONDON

Architecture of the cystic fibrosis transmembrane conductance regulator protein and structural changes associated with phosphorylation and nucleotide binding

(2009) Liang Zhang et al.   JOURNAL OF STRUCTURAL BIOLOGY

A Small-Molecule Modulator Interacts Directly with  Phe508-CFTR to Modify Its ATPase Activity and Conformational Stability

(2009) L. Wellhauser et al.   MOLECULAR PHARMACOLOGY

Strict coupling between CFTR's catalytic cycle and gating of its Cl- ion pore revealed by distributions of open channel burst durations

(2009) L. Csanady et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

(2009) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structure of P-Glycoprotein Reveals a Molecular Basis for Poly-Specific Drug Binding

(2009) S. G. Aller et al.   SCIENCE

The intact CFTR protein mediates ATPase rather than adenylate kinase activity

(2008) Mohabir Ramjeesingh et al.   BIOCHEMICAL JOURNAL

Solubilizing Mutations Used to Crystallize One CFTR Domain Attenuate the Trafficking and Channel Defects Caused by the Major Cystic Fibrosis Mutation

(2008) Luísa S. Pissarra et al.   CHEMISTRY & BIOLOGY

Computational Studies Reveal Phosphorylation-dependent Changes in the Unstructured R Domain of CFTR

(2008) Tamás Hegedűs et al.   JOURNAL OF MOLECULAR BIOLOGY

Regulation of Cl− secretion by AMPK in vivo

(2008) Patthara Kongsuphol et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

ATP hydrolysis-driven gating in cystic fibrosis transmembrane conductance regulator

(2008) D. Muallem et al.   PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES

Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function

(2008) A. W. R. Serohijos et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA