Unravelling druggable signalling networks that control F508del-CFTR proteostasis

Identification of p38 MAPK and JNK as new targets for correction of Wilson disease-causing ATP7B mutants

(2016) Giancarlo Chesi et al.   HEPATOLOGY

Ibuprofen rescues mutant cystic fibrosis transmembrane conductance regulator trafficking

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

RNA Interference Screen to Identify Kinases That Suppress Rescue of ΔF508-CFTR

(2015) Agata M. Trzcińska-Daneluti et al.   MOLECULAR & CELLULAR PROTEOMICS

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

(2015) Claire E. Wainwright et al.   NEW ENGLAND JOURNAL OF MEDICINE

Ferret and Pig Models of Cystic Fibrosis: Prospects and Promise for Gene Therapy

(2015) Ziying Yan et al.   Human Gene Therapy Clinical Development

Involvement of the Cdc42 Pathway in CFTR Post-Translational Turnover and in Its Plasma Membrane Stability in Airway Epithelial Cells

(2015) Romain Ferru-Clément et al.   PLoS One

Regulation of the Inflammasome by Ceramide in Cystic Fibrosis Lungs

(2014) Heike Grassmé et al.   CELLULAR PHYSIOLOGY AND BIOCHEMISTRY

Control Systems of Membrane Transport at the Interface between the Endoplasmic Reticulum and the Golgi

(2014) Jorge Cancino et al.   DEVELOPMENTAL CELL

MLK3 Is Part of a Feedback Mechanism That Regulates Different Cellular Responses to Reactive Oxygen Species

(2014) H.-S. Lee et al.   Science Signaling

Modulation of the Maladaptive Stress Response to Manage Diseases of Protein Folding

(2014) Daniela Martino Roth et al.   PLOS BIOLOGY

Deubiquitinases Sharpen Substrate Discrimination during Membrane Protein Degradation from the ER

(2013) Zai-Rong Zhang et al.   CELL

Revertants, Low Temperature, and Correctors Reveal the Mechanism of F508del-CFTR Rescue by VX-809 and Suggest Multiple Agents for Full Correction

(2013) Carlos M. Farinha et al.   CHEMISTRY & BIOLOGY

Targeting unfolded protein response signaling pathways to ameliorate protein misfolding diseases

(2013) Lisa M Ryno et al.   CURRENT OPINION IN CHEMICAL BIOLOGY

Discovery of novel potent ΔF508-CFTR correctors that target the nucleotide binding domain

(2013) Norbert Odolczyk et al.   EMBO Molecular Medicine

Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi

(2013) Carlos M. Farinha et al.   FEBS Journal

The Utility of Paradoxical Components in Biological Circuits

(2013) Yuval Hart et al.   MOLECULAR CELL

Characterization of drug-induced transcriptional modules: towards drug repositioning and functional understanding

(2013) M. Iskar et al.   Molecular Systems Biology

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

(2013) Tsukasa Okiyoneda et al.   Nature Chemical Biology

Tight Coordination of Protein Translation and HSF1 Activation Supports the Anabolic Malignant State

(2013) S. Santagata et al.   SCIENCE

Correction of F508del-CFTR Trafficking by the Sponge Alkaloid Latonduine Is Modulated by Interaction with PARP

(2012) Graeme W. Carlile et al.   CHEMISTRY & BIOLOGY

The KDEL receptor couples to Gαq/11to activate Src kinases and regulate transport through the Golgi

(2012) Monica Giannotta et al.   EMBO JOURNAL

Use of Kinase Inhibitors to Correct ΔF508-CFTR Function

(2012) Agata M. Trzcińska-Daneluti et al.   MOLECULAR & CELLULAR PROTEOMICS

RNAi screening reveals a large signaling network controlling the Golgi apparatus in human cells

(2012) Joanne Chia et al.   Molecular Systems Biology

Genome-wide RNAi screening identifies human proteins with a regulatory function in the early secretory pathway

(2012) Jeremy C. Simpson et al.   NATURE CELL BIOLOGY

STRING v9.1: protein-protein interaction networks, with increased coverage and integration

(2012) Andrea Franceschini et al.   NUCLEIC ACIDS RESEARCH

Multi-Target Drugs: The Trend of Drug Research and Development

(2012) Jin-Jian Lu et al.   PLoS One

Ouabain Mimics Low Temperature Rescue of F508del-CFTR in Cystic Fibrosis Epithelial Cells

(2012) Donglei Zhang et al.   Frontiers in Pharmacology

Decreasing Poly(ADP-Ribose) Polymerase Activity Restores ΔF508 CFTR Trafficking

(2012) Suzana M. Anjos et al.   Frontiers in Pharmacology

Identification of a NBD1-Binding Pharmacological Chaperone that Corrects the Trafficking Defect of F508del-CFTR

(2011) Heidi M. Sampson et al.   CHEMISTRY & BIOLOGY

Small-molecule proteostasis regulators for protein conformational diseases

(2011) Barbara Calamini et al.   Nature Chemical Biology

CFTR: folding, misfolding and correcting the ΔF508 conformational defect

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

Modifier genes in Mendelian disorders: the example of cystic fibrosis

(2010) Garry R. Cutting   Annals of the New York Academy of Sciences

Strategies for the identification of ubiquitin ligase inhibitors

(2010) Seth J. Goldenberg et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Loss of Cystic Fibrosis Transmembrane Conductance Regulator Function Enhances Activation of p38 and ERK MAPKs, Increasing Interleukin-6 Synthesis in Airway Epithelial Cells Exposed toPseudomonas aeruginosa

(2010) Julie Bérubé et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

MAPK signaling to the early secretory pathway revealed by kinase/phosphatase functional screening

(2010) Hesso Farhan et al.   JOURNAL OF CELL BIOLOGY

Small molecule correctors of F508del-CFTR discovered by structure-based virtual screening

(2010) Ori Kalid et al.   JOURNAL OF COMPUTER-AIDED MOLECULAR DESIGN

Correction of the  Phe508 Cystic Fibrosis Transmembrane Conductance Regulator Trafficking Defect by the Bioavailable Compound Glafenine

(2010) R. Robert et al.   MOLECULAR PHARMACOLOGY

Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition

(2010) Alessandro Luciani et al.   NATURE CELL BIOLOGY

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

Discovery of drug mode of action and drug repositioning from transcriptional responses

(2010) F. Iorio 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

Emergent Properties of Proteostasis in Managing Cystic Fibrosis

(2010) W. E. Balch et al.   Cold Spring Harbor Perspectives in Biology

The role of scaffold proteins in JNK signalling

(2009) W. Engström et al.   CELL PROLIFERATION

Differential Presentation of Protein Interaction Surfaces on the Androgen Receptor Defines the Pharmacological Actions of Bound Ligands

(2009) John David Norris et al.   CHEMISTRY & BIOLOGY

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

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

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

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

CFTR Function and Prospects for Therapy

(2008) John R. Riordan   Annual Review of Biochemistry

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

A traffic-activated Golgi-based signalling circuit coordinates the secretory pathway

(2008) Teodoro Pulvirenti et al.   NATURE CELL BIOLOGY