Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1

Regulatory Insertion Removal Restores Maturation, Stability and Function of ΔF508 CFTR

(2010) Andrei A. Aleksandrov et al.   JOURNAL OF MOLECULAR BIOLOGY

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

Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis

(2010) Chi Wang et al.   PROTEIN SCIENCE

Thermal stability of α-amylase in aqueous cosolvent systems

(2009) Jay Kant Yadav et al.   JOURNAL OF BIOSCIENCES

Structure and Dynamics of NBD1 from CFTR Characterized Using Crystallography and Hydrogen/Deuterium Exchange Mass Spectrometry

(2009) H.A. Lewis et al.   JOURNAL OF MOLECULAR BIOLOGY

Cooperative Assembly and Misfolding of CFTR Domains In Vivo

(2009) Kai Du et al.   MOLECULAR BIOLOGY OF THE CELL

CFTR Function and Prospects for Therapy

(2008) John R. Riordan   Annual Review of Biochemistry

Correctors promote folding of the CFTR in the endoplasmic reticulum

(2008) Tip W. Loo et al.   BIOCHEMICAL JOURNAL

Enhanced cell-surface stability of rescued ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones

(2008) Karoly Varga et al.   BIOCHEMICAL JOURNAL

Misfolding of the Cystic Fibrosis Transmembrane Conductance Regulator and Disease†

(2008) Joanne C. Cheung et al.   BIOCHEMISTRY

Thermodynamic and kinetic stability of penicillin acylase from Escherichia coli

(2008) Valerij Ya. Grinberg et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Atomic model of human cystic fibrosis transmembrane conductance regulator: Membrane-spanning domains and coupling interfaces

(2008) J.-P. Mornon et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

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

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

Chemical and Biological Folding Contribute to Temperature-Sensitive ΔF508 CFTR Trafficking

(2008) Xiaodong Wang et al.   TRAFFIC