Potentiation of ΔF508- and G551D-CFTR-Mediated Cl- Current by Novel Hydroxypyrazolines

Ibuprofen rescues mutant cystic fibrosis transmembrane conductance regulator trafficking

(2015) Graeme W. Carlile et al.   Journal of Cystic Fibrosis

Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells

(2014) Maria Favia et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

4-Functionalized 1,3-diarylpyrazoles bearing benzenesulfonamide moiety as selective potent inhibitors of the tumor associated carbonic anhydrase isoforms IX and XII

(2014) Poonam Khloya et al.   EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY

Some gating potentiators, including VX-770, diminish  F508-CFTR functional expression

(2014) G. Veit et al.   Science Translational Medicine

Potentiator ivacaftor abrogates pharmacological correction of  F508 CFTR in cystic fibrosis

(2014) D. M. Cholon et al.   Science Translational Medicine

Mechanism-based corrector combination restores ΔF508-CFTR folding and function

(2013) Tsukasa Okiyoneda et al.   Nature Chemical Biology

Thermal Instability of ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channel Function: Protection by Single Suppressor Mutations and Inhibiting Channel Activity

(2012) Xuehong Liu et al.   BIOCHEMISTRY

Novel 5-substituted benzyloxy-2-arylbenzofuran-3-carboxylic acids as calcium activated chloride channel inhibitors

(2012) Satish Kumar et al.   BIOORGANIC & MEDICINAL CHEMISTRY

Inhibitors of apoptosis in testicular germ cells: Synthesis and biological evaluation of some novel IBTs bearing sulfonamide moiety

(2012) Navneet Chandak et al.   EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY

Ivacaftor potentiation of multiple CFTR channels with gating mutations

(2012) Haihui Yu et al.   Journal of Cystic Fibrosis

Structure–Activity Relationships of Cyanoquinolines with Corrector–Potentiator Activity in ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator Protein

(2012) John M. Knapp et al.   JOURNAL OF MEDICINAL CHEMISTRY

Ivacaftor

(2012) Pamela B. Davis et al.   NATURE REVIEWS DRUG DISCOVERY

Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators

(2011) Louise C. Pyle et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Thermally Unstable Gating of the Most Common Cystic Fibrosis Mutant Channel (ΔF508)

(2011) Wei Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Synthesis of 1-(4-aminosulfonylphenyl)-3,5-diarylpyrazoline derivatives as potent antiinflammatory and antimicrobial agents

(2011) Pawan K. Sharma et al.   MEDICINAL CHEMISTRY RESEARCH

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

(2011) Bonnie W. Ramsey et al.   NEW ENGLAND JOURNAL OF MEDICINE

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

CFTR: folding, misfolding and correcting the ΔF508 conformational defect

(2011) Gergely L. Lukacs et al.   TRENDS IN MOLECULAR MEDICINE

Pyrazolylthiazole as ΔF508-Cystic Fibrosis Transmembrane Conductance Regulator Correctors with Improved Hydrophilicity Compared to Bithiazoles

(2010) Long Ye et al.   JOURNAL OF MEDICINAL CHEMISTRY

Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTRMutation

(2010) Frank J. Accurso et al.   NEW ENGLAND JOURNAL OF MEDICINE

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

(2009) L. Wellhauser et al.   MOLECULAR PHARMACOLOGY

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

CFTR Function and Prospects for Therapy

(2008) John R. Riordan   Annual Review of Biochemistry

Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease

(2008) Stan Pasyk et al.   BIOCHEMICAL JOURNAL

Potents-cis-Locked Bithiazole Correctors of ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator Cellular Processing for Cystic Fibrosis Therapy⊥

(2008) Gui Jun Yu et al.   JOURNAL OF MEDICINAL CHEMISTRY

Antimitotic and Antiproliferative Activities of Chalcones: Forward Structure–Activity Relationship

(2008) Ahcène Boumendjel et al.   JOURNAL OF MEDICINAL CHEMISTRY