The endoplasmic reticulum–associated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3–dependent degradation of nascent CFTRΔF508

The V510D Suppressor Mutation Stabilizes ΔF508-CFTR at the Cell Surface

(2010) Tip W. Loo et al.   BIOCHEMISTRY

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

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

The HSP70 chaperone machinery: J proteins as drivers of functional specificity

(2010) Harm H. Kampinga et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

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

N-glycans are direct determinants of CFTR folding and stability in secretory and endocytic membrane traffic

(2009) Rina Glozman et al.   JOURNAL OF CELL BIOLOGY

Mechanisms for Rescue of Correctable Folding Defects in CFTRΔF508

(2009) Diane E. Grove et al.   MOLECULAR BIOLOGY OF THE CELL

Cooperative Assembly and Misfolding of CFTR Domains In Vivo

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

The ubiquitylation machinery of the endoplasmic reticulum

(2009) Christian Hirsch et al.   NATURE

BAP31 Interacts with Sec61 Translocons and Promotes Retrotranslocation of CFTRΔF508 via the Derlin-1 Complex

(2008) Bing Wang et al.   CELL

Dissecting the ER-Associated Degradation of a Misfolded Polytopic Membrane Protein

(2008) Kunio Nakatsukasa et al.   CELL

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

Gp78 Cooperates with RMA1 in Endoplasmic Reticulum-associated Degradation of CFTRΔF508

(2008) Daisuke Morito et al.   MOLECULAR BIOLOGY OF THE CELL

Assembly and Misassembly of Cystic Fibrosis Transmembrane Conductance Regulator: Folding Defects Caused by Deletion of F508 Occur Before and After the Calnexin-dependent Association of Membrane Spanning Domain (MSD) 1 and MSD2

(2008) Meredith F. N. Rosser et al.   MOLECULAR BIOLOGY OF THE CELL

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

Diminished Self-Chaperoning Activity of the ΔF508 Mutant of CFTR Results in Protein Misfolding

(2008) Adrian W. R. Serohijos et al.   PLoS Computational Biology