Mice, American Journal of Respiratory Cell and Molecular Biology, vol.40, issue.3, pp.295-304, 2009. ,
DOI : 10.1165/rcmb.2008-0170OC
Abnormal Trafficking and Degradation of TLR4 Underlie the Elevated Inflammatory Response in Cystic Fibrosis, The Journal of Immunology, vol.186, issue.12, pp.6990-6998, 2011. ,
DOI : 10.4049/jimmunol.1100396
mutation, Thorax, vol.67, issue.1, pp.12-18, 2012. ,
DOI : 10.1136/thoraxjnl-2011-200393
Canonical and non-canonical autophagy: variations on a common theme of self-eating?, Nature Reviews Molecular Cell Biology, vol.7, pp.7-12, 2011. ,
DOI : 10.4161/auto.7.11.17661
Cystic Fibrosis Since 1938, American Journal of Respiratory and Critical Care Medicine, vol.173, issue.5, pp.475-482, 2006. ,
DOI : 10.1164/rccm.200505-840OE
Therapy for Cystic Fibrosis ??? The End of the Beginning?, New England Journal of Medicine, vol.365, issue.18, pp.1734-1735, 2011. ,
DOI : 10.1056/NEJMe1110323
Emerging roles of ppars in inflammation and immunity, Nature Reviews Immunology, vol.2, issue.10, pp.748-759, 2002. ,
DOI : 10.1038/nri912
Dysfunctional CFTR Alters the Bactericidal Activity of Human Macrophages against Pseudomonas aeruginosa, PLoS ONE, vol.23, issue.5, 2011. ,
DOI : 10.1371/journal.pone.0019970.t001
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive, Nature, vol.358, issue.6389, pp.761-764, 1992. ,
DOI : 10.1038/358761a0
Aggregopathy in Neurodegenerative Diseases: Mechanisms and Therapeutic Implication, Neurodegenerative Diseases, vol.5, issue.6, pp.321-338, 2008. ,
DOI : 10.1159/000119459
p62 Is a Key Regulator of Nutrient Sensing in the mTORC1 Pathway, Molecular Cell, vol.44, issue.1, pp.134-146, 2011. ,
DOI : 10.1016/j.molcel.2011.06.038
Curcumin, a Major Constituent of Turmeric, Corrects Cystic Fibrosis Defects, Science, vol.304, issue.5670, pp.600-602, 2004. ,
DOI : 10.1126/science.1093941
Fixing cystic fibrosis CFTR with correctors and potentiators. Off to a good start, Thorax, vol.67, issue.1, pp.4-5, 2012. ,
DOI : 10.1136/thoraxjnl-2011-201197
Novel CFTR Chloride Channel Activators Identified by Screening of Combinatorial Libraries Based on Flavone and Benzoquinolizinium Lead Compounds, Journal of Biological Chemistry, vol.279, issue.23, 2001. ,
DOI : 10.1093/jnci/82.13.1113
Trafficking via a GRASP-dependent unconventional secretion pathway, Cell, vol.146, pp.746-760 ,
Concepts in sumoylation: a decade on, Nature Reviews Molecular Cell Biology, vol.1773, issue.12, pp.947-956, 2007. ,
DOI : 10.1016/j.cub.2004.02.004
A cellular perspective on conformational disease: the role of genetic background and proteostasis networks, Current Opinion in Structural Biology, vol.20, issue.1, pp.23-32, 2010. ,
DOI : 10.1016/j.sbi.2009.11.001
A subdomain of the endoplasmic reticulum forms a cradle for autophagosome formation, Nature Cell Biology, vol.4, issue.12, pp.1433-1437, 2009. ,
DOI : 10.1091/mbc.E07-08-0781
The Beclin 1 interactome, Current Opinion in Cell Biology, vol.22, issue.2, pp.140-149, 2010. ,
DOI : 10.1016/j.ceb.2010.01.001
The Delta F508 mutation shortens the biochemical halflife of plasmamembrane CFTR in polarized epithelial cells, Am. J. Physiol . Cell Physiol, vol.280, pp.166-174, 2001. ,
Expression of wild-type CFTR suppresses NF-kappaB-driven inflammatory signalling, PLoS ONE, vol.5, 2010. ,
The Proteome in Balance, Science, vol.21, issue.2, pp.766-770, 2010. ,
DOI : 10.1101/gad.1505307
The proteostasis boundary in misfolding diseases of membrane traffic, FEBS Letters, vol.21, issue.16, pp.2639-2646, 2009. ,
DOI : 10.1101/gad.1505307
Transglutaminases and Disease: Lessons From Genetically Engineered Mouse Models and Inherited Disorders, Physiological Reviews, vol.89, issue.3, pp.991-1023, 2009. ,
DOI : 10.1152/physrev.00044.2008
NF-??B: linking inflammation and immunity to cancer development and progression, Nature Reviews Immunology, vol.100, issue.10, pp.749-759, 2005. ,
DOI : 10.1016/S0092-8674(00)81683-9
Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine, Nature Medicine, vol.8, issue.2, pp.143-149, 2002. ,
DOI : 10.1038/nm0202-143
The Deacetylase HDAC6 Regulates Aggresome Formation and Cell Viability in Response to Misfolded Protein Stress, Cell, vol.115, issue.6, pp.727-738, 2003. ,
DOI : 10.1016/S0092-8674(03)00939-5
Reversal of Drug Resistance in Breast Cancer Cells by Transglutaminase 2 Inhibition and Nuclear Factor-??B Inactivation, Cancer Research, vol.66, issue.22, pp.10936-10943, 2006. ,
DOI : 10.1158/0008-5472.CAN-06-1521
A Role for Ubiquitin in Selective Autophagy, Molecular Cell, vol.34, issue.3, pp.259-269, 2009. ,
DOI : 10.1016/j.molcel.2009.04.026
Homeostatic Levels of p62 Control Cytoplasmic Inclusion Body Formation in Autophagy-Deficient Mice, Cell, vol.131, issue.6, pp.1149-1163, 2007. ,
DOI : 10.1016/j.cell.2007.10.035
Autophagy Inhibition Compromises Degradation of Ubiquitin-Proteasome Pathway Substrates, Molecular Cell, vol.33, issue.4, pp.517-527, 2009. ,
DOI : 10.1016/j.molcel.2009.01.021
Autophagy and the Integrated Stress Response, Molecular Cell, vol.40, issue.2, pp.280-293, 2010. ,
DOI : 10.1016/j.molcel.2010.09.023
URL : http://doi.org/10.1016/j.molcel.2010.09.023
Autophagy in immunity and inflammation, Nature, vol.307, issue.7330, pp.323-335, 2011. ,
DOI : 10.1126/science.1106036
Beclin1-binding UVRAG targets the class C Vps complex to coordinate autophagosome maturation and endocytic trafficking, Nature Cell Biology, vol.69, issue.7, pp.776-787, 2008. ,
DOI : 10.1074/jbc.271.45.28593
Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition, Nature Cell Biology, vol.59, issue.9, pp.863-875, 2010. ,
DOI : 10.4161/auto.5338
Cystic fibrosis: A disorder with defective autophagy, Autophagy, vol.7, issue.1, pp.104-106, 2011. ,
DOI : 10.4161/auto.7.1.13987
Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ??F508 cystic fibrosis transmembrane conductance regulator, Autophagy, vol.14, issue.11, pp.1657-1672, 2012. ,
DOI : 10.1073/pnas.1105787108
The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells, 1993. ,
PIASy mediates NEMO sumoylation and NF-??B activation in response to genotoxic stress, Nature Cell Biology, vol.18, issue.9, pp.986-993, 2006. ,
DOI : 10.1128/MCB.24.11.4895-4908.2004
Tissue Transglutaminase Activation Modulates Inflammation in Cystic Fibrosis via PPAR?? Down-Regulation, The Journal of Immunology, vol.180, issue.11, pp.7697-7705, 2008. ,
DOI : 10.4049/jimmunol.180.11.7697
URL : http://citeseerx.ist.psu.edu/viewdoc/summary?doi=10.1.1.318.2294
Crosstalk between apoptosis and autophagy within the Beclin 1 interactome, The EMBO Journal, vol.29, issue.3, pp.515-516, 2010. ,
DOI : 10.1038/ncb1854
URL : http://emboj.embopress.org/content/embojnl/29/3/515.full.pdf
Functional and physical interaction between Bcl-XL and a BH3-like domain in Beclin-1, The EMBO Journal, vol.15, issue.10, pp.2527-2539, 2007. ,
DOI : 10.1126/stke.2003.173.pe10
Type 2 transglutaminasein neurodegenerative diseases: the mitochondrial connection, Curr. Pharm. Des, vol.14, pp.278-288, 2008. ,
Store Expansion Is Mediated by X-box Binding Protein-1, Journal of Biological Chemistry, vol.8, issue.22, pp.14904-14913, 2009. ,
DOI : 10.1073/pnas.0509487102
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2685672
Type 2 transglutaminase in Huntington's disease: a doubleedged sword with clinical potential, 2010. ,
Autophagy Suppresses Tumorigenesis through Elimination of p62, Cell, vol.137, issue.6, pp.1062-1075, 2009. ,
DOI : 10.1016/j.cell.2009.03.048
Two Beclin 1-binding proteins, Atg14L and Rubicon, reciprocally regulate autophagy at different stages, Nature Cell Biology, vol.19, issue.4, pp.385-396, 2009. ,
DOI : 10.1128/MCB.18.9.5308
Cell biology: SUMO, Nature, vol.452, issue.7188, pp.709-711, 2008. ,
DOI : 10.1038/452709a
The Role of Atg Proteins in Autophagosome Formation, Annual Review of Cell and Developmental Biology, vol.27, issue.1, pp.107-132, 2011. ,
DOI : 10.1146/annurev-cellbio-092910-154005
Cytoprotective roles for autophagy, Current Opinion in Cell Biology, vol.22, issue.2, pp.206-211, 2010. ,
DOI : 10.1016/j.ceb.2009.12.002
URL : http://doi.org/10.1016/j.ceb.2009.12.002
One-year glargine treatment can improve the course of lung disease in children and adolescents with cystic fibrosis and early glucose derangements, Pediatric Diabetes, vol.147, issue.Suppl. 2, pp.162-167, 2009. ,
DOI : 10.1007/BF03345538
Chemical and Biological Approaches Synergize to Ameliorate Protein-Folding Diseases, Cell, vol.134, issue.5, pp.769-781, 2008. ,
DOI : 10.1016/j.cell.2008.06.037
URL : http://doi.org/10.1016/j.cell.2008.06.037
Transglutaminase Is Linked to Neurodegenerative Diseases, Journal of Neuropathology & Experimental Neurology, vol.66, issue.4, pp.258-263, 2007. ,
DOI : 10.1097/nen.0b013e31803d3b02
URL : https://academic.oup.com/jnen/article-pdf/66/4/258/9556462/66-4-258.pdf
Maintaining Low Ca2+ Level in the Endoplasmic Reticulum Restores Abnormal Endogenous F508del-CFTR Trafficking in Airway Epithelial Cells, Traffic, vol.260, issue.Suppl. 2, pp.562-573, 2006. ,
DOI : 10.1172/JCI0216112
URL : https://hal.archives-ouvertes.fr/hal-00078138
Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the ??-glucosidase inhibitor miglustat, FEBS Letters, vol.5, issue.8, pp.2081-2086, 2006. ,
DOI : 10.1038/4801
Cellular Functions of Tissue Transglutaminase, Int. Rev. Cell. Mol. Biol, vol.294, pp.1-97, 2012. ,
DOI : 10.1016/B978-0-12-394305-7.00001-X
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3746560
Protein quality control at the plasma membrane, Current Opinion in Cell Biology, vol.23, issue.4, pp.483-491, 2011. ,
DOI : 10.1016/j.ceb.2011.04.012
Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane, Science, vol.284, issue.28, pp.805-810, 2010. ,
DOI : 10.1074/jbc.M109.001685
Cystic fibrosis, The Lancet, vol.373, issue.9678, pp.1891-1904, 2009. ,
DOI : 10.1016/S0140-6736(09)60327-5
Dynamic Regulation of CFTR Bicarbonate Permeability by [Cl???]i and Its Role in Pancreatic Bicarbonate Secretion, Gastroenterology, vol.139, issue.2, pp.620-631, 2010. ,
DOI : 10.1053/j.gastro.2010.04.004
A SUMOylation-dependent pathway mediates transrepression of inflammatory response genes by PPAR-??, Nature, vol.28, issue.7059, pp.759-763, 2005. ,
DOI : 10.1038/35056591
Regulation and Functional Significance of Autophagy in Respiratory Cell Biology and Disease, American Journal of Respiratory Cell and Molecular Biology, vol.48, issue.1, 2013. ,
DOI : 10.1165/rcmb.2012-0282TR
Bcl-2 Antiapoptotic Proteins Inhibit Beclin 1-Dependent Autophagy, Cell, vol.122, issue.6, pp.927-939, 2005. ,
DOI : 10.1016/j.cell.2005.07.002
URL : http://doi.org/10.1016/j.cell.2005.07.002
Small-molecule correctors of defective ??F508-CFTR cellular processing identified by high-throughput screening, Journal of Clinical Investigation, vol.115, issue.9, pp.2564-2571, 2005. ,
DOI : 10.1172/JCI24898DS1
Biological and Chemical Approaches to Diseases of Proteostasis Deficiency, Annual Review of Biochemistry, vol.78, issue.1, pp.959-991, 2009. ,
DOI : 10.1146/annurev.biochem.052308.114844
Physiological basis of cystic fibrosis: a historical perspective, Physiol. Rev, vol.79, pp.3-22, 1999. ,
Inhibition of p38 mitogen activated protein kinase controls airway inflammation in cystic fibrosis, Thorax, vol.60, issue.9, pp.773-780, 2005. ,
DOI : 10.1136/thx.2005.042564
Mutation, New England Journal of Medicine, vol.365, issue.18, pp.1663-1672, 2011. ,
DOI : 10.1056/NEJMoa1105185
New Therapies in Cystic Fibrosis, Current Pharmaceutical Design, vol.18, issue.5, pp.614-627, 2012. ,
DOI : 10.2174/138161212799315984
Cystic Fibrosis: Pathogenesis and Future Treatment Strategies, Respiratory Care, vol.54, issue.5, pp.595-605, 2009. ,
DOI : 10.4187/aarc0427
Signaling, Journal of Biological Chemistry, vol.17, issue.11, pp.10202-10209, 2005. ,
DOI : 10.1083/jcb.149.4.793
URL : https://hal.archives-ouvertes.fr/hal-01369518
-dependent Hyperinflammatory Response in Human Cystic Fibrosis Airway Epithelia, Journal of Biological Chemistry, vol.31, issue.18, pp.17798-17806, 2005. ,
DOI : 10.1099/00221287-144-8-2037
URL : https://hal.archives-ouvertes.fr/jpa-00251166
CFTR Function and Prospects for Therapy, Annual Review of Biochemistry, vol.77, issue.1, pp.701-726, 2008. ,
DOI : 10.1146/annurev.biochem.75.103004.142532
Modeling general proteostasis: proteome balance in health and disease, Current Opinion in Cell Biology, vol.23, issue.2, pp.126-134, 2011. ,
DOI : 10.1016/j.ceb.2010.11.001
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3077458
Enhanced Peritoneal Ovarian Tumor Dissemination by Tissue Transglutaminase, Cancer Research, vol.67, issue.15, pp.7194-7202, 2007. ,
DOI : 10.1158/0008-5472.CAN-07-0307
Misfolding diverts CFTR from recycling to degradation, The Journal of Cell Biology, vol.15, issue.6, pp.923-933, 2004. ,
DOI : 10.1146/annurev.ge.29.120195.004021
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2172283
Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis, Current Opinion in Pulmonary Medicine, vol.16, issue.6, pp.591-597, 2010. ,
DOI : 10.1097/MCP.0b013e32833f1d00
Autophagy and disease: always two sides to a problem, The Journal of Pathology, vol.5, issue.suppl 2, pp.255-273, 2012. ,
DOI : 10.1016/j.chom.2009.05.016
URL : http://onlinelibrary.wiley.com/doi/10.1002/path.3025/pdf
Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein, Human Molecular Genetics, vol.12, issue.7, pp.749-757, 2003. ,
DOI : 10.1093/hmg/ddg074
SUMO under stress: Figure 1, Biochemical Society Transactions, vol.36, issue.5, pp.874-878, 2008. ,
DOI : 10.1042/BST0360874
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809, Proc. Natl. Acad, 2011. ,
DOI : 10.1172/JCI24898
Rescue of DeltaF508- CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules, 2006. ,
Chloride channels as drug targets, Nature Reviews Drug Discovery, vol.19, issue.2, pp.153-171, 2009. ,
DOI : 10.1007/s11068-008-9026-7
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3601949
CFTR Chloride Channel Drug Discovery - Inhibitors as Antidiarrheals and Activators for Therapy of Cystic Fibrosis, Current Pharmaceutical Design, vol.12, issue.18, pp.2235-2247, 2006. ,
DOI : 10.2174/138161206777585148
CFTR is a negative regulator of NFkappa B mediated innate immune response, PLoS ONE, vol.4, 2009. ,
Degradation of CFTR by the ubiquitin-proteasome pathway, Cell, vol.83, issue.1, pp.121-127, 1995. ,
DOI : 10.1016/0092-8674(95)90240-6
The metabolic basis of inherited disease, pp.5121-5188, 2001. ,
Eaten alive: a history of macroautophagy, Nature Cell Biology, vol.7, issue.9, pp.814-822, 2010. ,
DOI : 10.1038/ncb0910-823
Alterations in ceramide concentration and pH determine the release of reactive oxygen species by Cftrdeficient macrophages on infection, 2010. ,
Distinct regulation of autophagic activity by Atg14L and Rubicon associated with Beclin 1???phosphatidylinositol-3-kinase complex, Nature Cell Biology, vol.104, issue.4, pp.468-476, 2009. ,
DOI : 10.4161/auto.3602