Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator

Influence of cell background on pharmacological rescue of mutant CFTR

(2010) Nicoletta Pedemonte et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

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

Syntaxin 6 and CAL Mediate the Degradation of the Cystic Fibrosis Transmembrane Conductance Regulator

(2010) Jie Cheng et al.   MOLECULAR BIOLOGY OF THE CELL

ΔF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers

(2010) S.M. Rowe et al.   PULMONARY PHARMACOLOGY & THERAPEUTICS

Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures

(2009) Deborah M. Cholon et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Functional Stability of Rescued ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator in Airway Epithelial Cells

(2009) Asta Jurkuvenaite et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Emerging Treatments in Cystic Fibrosis

(2009) Andrew M. Jones et al.   DRUGS

The ER-resident ubiquitin-specific protease 19 participates in the UPR and rescues ERAD substrates

(2009) Gerco C Hassink et al.   EMBO REPORTS

Airway Epithelial Inflammation-induced Endoplasmic Reticulum Ca2+Store Expansion Is Mediated by X-box Binding Protein-1

(2009) Mary E. B. Martino et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Rescue of ΔF508-CFTR by the SGK1/Nedd4-2 Signaling Pathway

(2009) Hung Caohuy et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mechanisms for Rescue of Correctable Folding Defects in CFTRΔF508

(2009) Diane E. Grove 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

Na+/H+ Exchanger Regulatory Factor 1 Overexpression-dependent Increase of Cytoskeleton Organization Is Fundamental in the Rescue of F508del Cystic Fibrosis Transmembrane Conductance Regulator in Human Airway CFBE41o- Cells

(2009) Maria Favia et al.   MOLECULAR BIOLOGY OF THE CELL

Partial Correction of Cystic Fibrosis Defects with PLGA Nanoparticles Encapsulating Curcumin

(2009) Malgorzata S. Cartiera et al.   MOLECULAR PHARMACEUTICS

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

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

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

Cystic fibrosis gene therapy: successes, failures and hopes for the future

(2009) Uta Griesenbach et al.   Expert Review of Respiratory Medicine

Role of Oxygen Availability in CFTR Expression and Function

(2008) Jennifer S. Guimbellot et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Activation of the Unfolded Protein Response by ΔF508 CFTR

(2008) Rafal Bartoszewski et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

CFTR Function and Prospects for Therapy

(2008) John R. Riordan   Annual Review of Biochemistry

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

(2008) Karoly Varga et al.   BIOCHEMICAL JOURNAL

Cigarette smoke induces endoplasmic reticulum stress and the unfolded protein response in normal and malignant human lung cells

(2008) Ellen Jorgensen et al.   BMC CANCER

The Mechanism of Cystic Fibrosis Transmembrane Conductance Regulator Transcriptional Repression during the Unfolded Protein Response

(2008) Rafal Bartoszewski et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial

(2008) Eitan Kerem et al.   LANCET

High-content Functional Screen to Identify Proteins that Correct F508del-CFTR Function

(2008) Agata M. Trzcińska-Daneluti et al.   MOLECULAR & CELLULAR PROTEOMICS

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

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

Hypoxic Inhibition of Nonsense-Mediated RNA Decay Regulates Gene Expression and the Integrated Stress Response

(2008) L. B. Gardner   MOLECULAR AND CELLULAR BIOLOGY

From endoplasmic-reticulum stress to the inflammatory response

(2008) Kezhong Zhang et al.   NATURE

PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model

(2008) M. Du 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

Introducing sense into nonsense in treatments of human genetic diseases

(2008) Liat Linde et al.   TRENDS IN GENETICS