Human Heat Shock Protein 105/110 kDa (Hsp105/110) Regulates Biogenesis and Quality Control of Misfolded Cystic Fibrosis Transmembrane Conductance Regulator at Multiple Levels

FKBP38 Peptidylprolyl Isomerase Promotes the Folding of Cystic Fibrosis Transmembrane Conductance Regulator in the Endoplasmic Reticulum

(2011) Yeshavanth K. Banasavadi-Siddegowda et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809

(2011) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for theF508del-CFTRmutation

(2011) J P Clancy et al.   THORAX

Hsp105 reduces the protein aggregation and cytotoxicity by expanded-polyglutamine proteins through the induction of Hsp70

(2010) Nobuyuki Yamagishi et al.   EXPERIMENTAL CELL RESEARCH

Restoration of domain folding and interdomain assembly by second-site suppressors of the ΔF508 mutation in CFTR

(2010) Lihua He et al.   FASEB JOURNAL

Ubiquitin C-terminal Hydrolase-L1 Protects Cystic Fibrosis Transmembrane Conductance Regulator from Early Stages of Proteasomal Degradation

(2010) Mark J. Henderson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp110 Chaperones Control Client Fate Determination in the Hsp70–Hsp90 Chaperone System

(2010) Atin K. Mandal et al.   MOLECULAR BIOLOGY OF THE CELL

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

(2010) Diane E. Grove et al.   MOLECULAR BIOLOGY OF THE CELL

Hsp 70/Hsp 90 organizing protein as a nitrosylation target in cystic fibrosis therapy

(2010) N. V. Marozkina et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane

(2010) T. Okiyoneda et al.   SCIENCE

Cooperative Assembly and Misfolding of CFTR Domains In Vivo

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

Interplay between ER Exit Code and Domain Conformation in CFTR Misprocessing and Rescue

(2009) Gargi Roy et al.   MOLECULAR BIOLOGY OF THE CELL

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

(2009) Darren M Hutt et al.   Nature Chemical Biology

Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia

(2008) Fei Sun et al.   FASEB JOURNAL

Cystic Fibrosis Transmembrane Regulator Missing the First Four Transmembrane Segments Increases Wild Type and ΔF508 Processing

(2008) Liudmila Cebotaru et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Chemical Rescue of ΔF508-CFTR Mimics Genetic Repair in Cystic Fibrosis Bronchial Epithelial Cells

(2008) Om V. Singh et al.   MOLECULAR & CELLULAR PROTEOMICS

Structure of the Hsp110:Hsc70 Nucleotide Exchange Machine

(2008) Jonathan P. Schuermann et al.   MOLECULAR CELL

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

(2008) Xiaodong Wang et al.   TRAFFIC