Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ΔF508 cystic fibrosis transmembrane conductance regulator

Tissue Transglutaminase Activation Modulates Inflammation in Cystic Fibrosis via PPAR  Down-Regulation

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New Therapies in Cystic Fibrosis

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The Role of Atg Proteins in Autophagosome Formation

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Autophagy stimulation by rapamycin suppresses lung inflammation and infection byBurkholderia cenocepaciain a model of cystic fibrosis

(2011) Basant A. Abdulrahman et al.   Autophagy

Targeting CFTR: How to Treat Cystic Fibrosis by CFTR-Repairing Therapies

(2011) Margarida D. Amaral   CURRENT DRUG TARGETS

Protein quality control at the plasma membrane

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Mouse models of cystic fibrosis: Phenotypic analysis and research applications

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Autophagy and disease: always two sides to a problem

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Autophagy in immunity and inflammation

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Canonical and non-canonical autophagy: variations on a common theme of self-eating?

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Therapy for Cystic Fibrosis — The End of the Beginning?

(2011) Pamela B. Davis   NEW ENGLAND JOURNAL OF MEDICINE

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

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

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Fixing cystic fibrosis CFTR with correctors and potentiators. Off to a good start

(2011) J Stuart Elborn   THORAX

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

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CFTR: folding, misfolding and correcting the ΔF508 conformational defect

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Cystic fibrosis: A disorder with defective autophagy

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Protein Processing and Inflammatory Signaling in Cystic Fibrosis: Challenges and Therapeutic Strategies

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A cellular perspective on conformational disease: the role of genetic background and proteostasis networks

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Dynamic Regulation of CFTR Bicarbonate Permeability by [Cl−]i and Its Role in Pancreatic Bicarbonate Secretion

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Autophagy and the Integrated Stress Response

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A Chemical Corrector Modifies the Channel Function of F508del-CFTR

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Eaten alive: a history of macroautophagy

(2010) Zhifen Yang et al.   NATURE CELL BIOLOGY

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

(2010) Alessandro Luciani et al.   NATURE CELL BIOLOGY

Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane

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Emergent Properties of Proteostasis in Managing Cystic Fibrosis

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Emerging therapies in cystic fibrosis

(2010) Paula Anderson   Therapeutic Advances in Respiratory Disease

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

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p62 at the Crossroads of Autophagy, Apoptosis, and Cancer

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Lysosomal accumulation of gliadin p31-43 peptide induces oxidative stress and tissue transglutaminase-mediated PPAR  downregulation in intestinal epithelial cells and coeliac mucosa

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

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SUMOylation of Tissue Transglutaminase as Link between Oxidative Stress and Inflammation

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Cystic fibrosis

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Rab11b Regulates the Apical Recycling of the Cystic Fibrosis Transmembrane Conductance Regulator in Polarized Intestinal Epithelial Cells

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Chloride channels as drug targets

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One-year glargine treatment can improve the course of lung disease in children and adolescents with cystic fibrosis and early glucose derangements

(2009) Enza Mozzillo et al.   PEDIATRIC DIABETES

CFTR Is a Negative Regulator of NFκB Mediated Innate Immune Response

(2009) Neeraj Vij et al.   PLoS One

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

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

Feasibility of nasal epithelial brushing for the study of airway epithelial functions in CF infants

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