Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA, Science, vol.245, pp.1066-1073, 1989. ,
Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR), Cell, vol.68, pp.809-818, 1992. ,
Gating of the CFTR Cl ? channel by ATP-driven nucleotide-binding domain dimerisation, J Physiol, vol.587, pp.2151-2161, 2009. ,
Processing and function of CFTR-?F508 are species-dependent, Proc Natl Acad Sci, vol.104, pp.15370-15375, 2007. ,
Potentiation of disease-associated cystic fibrosis transmembrane conductance regulator mutants by hydrolyzable ATP analogs, J Biol Chem, vol.285, 2010. ,
CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains, Nature, vol.433, pp.876-880, 2005. ,
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR, J. Clin. Invest, vol.100, pp.2457-2465, 1997. ,
Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein, Cell Stress Chaperones, vol.1, pp.117-125, 1996. ,
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive, Nature, vol.358, pp.761-764, 1992. ,
Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat, FEBS Lett, vol.580, pp.2081-2086, 2006. ,
Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones, Mol. Pharmacol, vol.70, pp.297-302, 2006. ,
Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein, J. Biol. Chem, vol.282, pp.33247-33251, 2007. ,
Development of substituted Benzo [c]quinolizinium compounds as novel activators of the cystic fibrosis chloride channel, J. Biol. Chem, vol.274, pp.27415-27425, 1999. ,
Annexin V is directly involved in cystic fibrosis transmembrane conductance regulator's chloride channel function, Biochim Biophys Acta, vol.1772, pp.1121-1133, 2007. ,
Annexin A5 increases the cell surface expression and the chloride channel function of the ?F508-Cystic Fibrosis Transmembrane Regulator, Biochim Biophys Acta, vol.1782, pp.605-614, 2008. ,
Function and regulation of TRPM7, as well as intracellular magnesium content, are altered in cells expressing ?F508-CFTR and G551D-CFTR, Cellular and Molecular Life Sciences, vol.73, issue.17, pp.3351-73, 2016. ,
Cystic fibrosis: genotypic and phenotypic variations, Annu Rev Genet, vol.29, pp.777-807, 1995. ,
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis, Cell, vol.63, pp.827-834, 1990. ,
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation, J Biol Chem, vol.271, pp.635-638, 1996. ,
Dysfunction of CFTR bearing the delta F508 mutation, J Cell Sci, vol.17, pp.235-239, 1993. ,
Altered chloride ion channel kinetics associated with the DF508 cystic fibrosis mutation, Nature, vol.354, pp.526-528, 1991. ,
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, J Biol Chem, vol.268, pp.21592-21598, 1993. ,
, Cell Mol Life Sci, 2016.
Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening, J Clin Invest, vol.115, pp.2564-2571, 2005. ,
Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat, FEBS Lett, 2006. ,
, , vol.580, pp.2081-2086
Correction of the phe508 cystic fibrosis transmembrane conductance regulator trafficking defect by the bioavailable compound glafenine, Mol Pharmacol, vol.77, pp.922-930, 2010. ,
Correction of F508del-CFTR trafficking by the sponge alkaloid latonduine is modulated by interaction with PARP, Chem Biol, vol.19, pp.1288-1299, 2012. ,
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809, Proc Natl Acad Sci, vol.108, pp.18843-18848, 2011. ,
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation, Thorax, vol.67, pp.12-18, 2012. ,
Potentiator ivacaftor abrogates pharmacological correction of delF508 CFTR in cystic fibrosis, Sci Transl Med, vol.6, pp.246-96, 2014. ,
Improvement of Chloride Transport Defect by Gonadotropin-Releasing Hormone (GnRH) in Cystic Fibrosis Epithelial Cells, PLoSONE, vol.9, issue.2, p.88964, 2014. ,
URL : https://hal.archives-ouvertes.fr/hal-00990343
Cystic fibrosis airway epithelium remodelling: involvement of inflammation, J Pathol, vol.235, issue.3, pp.408-419, 2015. ,
Automated ion channel screening: patch clamping made easy, Expert Opin Ther Targets, vol.11, issue.4, pp.557-565, 2007. ,
Sensitivity of pituitary and corpus luteum responses to single intranasal administration of (D-ser[TBU]6-des-gly-NH2(10)) luteinizing hormone-releasing hormone ethylamide (Buserelin) in normal women, Fertil Steril, vol.37, issue.2, pp.193-200, 1982. ,
Expression of gonadotropin-releasing hormone and gonadotropin-releasing hormone receptor mRNAs in various non-reproductive human tissues, Cancer Lett, vol.98, issue.1, pp.57-62, 1995. ,
Gonadotropin-releasing hormone receptor expression in the human prostate, Prostate, vol.47, issue.4, pp.276-284, 2001. ,
Evidence for LHRH-Receptor Expression in Human Airway Epithelial (Calu-3) Cells and Its Role in the Transport of a LHRH Agonist, Pharmaceutical Research, vol.21, issue.6, pp.1034-1046, 2004. ,
Cl transport in complemented CF bronchial epithelial cells correlates with CFTR mRNA expression levels, Cell. Physiol. Biochem, vol.22, pp.57-68, 2008. ,
Mucin gene expression during differentiation of human airway epithelia in vitro. Muc4 and muc5b are strongly induced, Am J Respir Cell Mol Biol, vol.20, issue.4, pp.595-604, 1999. ,
Label-free whole-cell assays: expanding the scope of GPCR screening, Drug. Discov. Today, vol.15, pp.704-716, 2010. ,
Persistent GnRH receptor activation in pituitary ?T3-1 cells analyzed with a label-free technology, Biosens Bioelectron, vol.15, issue.79, pp.721-727, 2016. ,
Characterization of 12 GnRH peptide agonists-a kinetic perspective, J. Pharmacol, vol.173, pp.128-141, 2015. ,
Characterization of 12 GnRH peptide agonists-a kinetic perspective, Br J Pharmacol, vol.173, issue.1, pp.128-169, 2016. ,
Pathways of degradation of buserelin by rat kidney membrane, Journal of Pharmacology and Experimental Therapeutics, vol.273, issue.2, pp.709-715, 1995. ,
Molecular Mechanisms of Gonadotropin-Releasing Hormone Signaling: Integrating Cyclic Nucleotides into the Network, Front Endocrinol, vol.4, p.180, 2013. ,
Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis, Physiol. Rev, vol.79, pp.77-107, 1999. ,
Synergy of cAMP and calcium signaling pathways in CFTR regulation, Proc Natl Acad Sci, vol.114, issue.11, pp.2086-2095, 2017. ,
Serpin structure, mechanism, and function, Chem Rev, vol.102, issue.12, pp.4751-4804, 2002. ,
Serpins flex their muscle: I. Putting the clamps on proteolysis in diverse biological systems, J Biol Chem, 2010. ,
, , vol.285, pp.24299-24305
SERPINB3 protects from oxidative damage by chemotherapeutics through inhibition of mitochondrial respiratory complex I, vol.5, pp.2418-2427, 2014. ,
SERPINB3 and B4: From biochemistry to biology, Semin Cell Dev Biol, vol.62, pp.170-177, 2017. ,
Overexpression of SERPIN B3 promotes epithelial proliferation and lung fibrosis in mice, Lab Invest, vol.91, issue.6, pp.945-54, 2011. ,
Overexpression of squamous cell carcinoma antigen in idiopathic pulmonary fibrosis: clinicopathological correlations, Thorax, vol.63, issue.9, pp.795-802, 2008. ,
The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function, PLoSOne, vol.7, issue.11, p.48059, 2012. ,
VDAC2-specific cellular functions and the underlying structure, BBA-Molecular Cell Research, vol.1863, pp.2503-2514, 2016. ,
CFTR activity and mitochondrial function, Redox Biol, vol.1, pp.190-202, 2013. ,
Characterization of mitochondrial function in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function, J Bioenerg Biomembr, vol.48, issue.3, p.27146408, 2016. ,
CFTR and chaperones: processing and degradation, J Mol Neurosci, vol.23, pp.41-48, 2004. ,
Chemical Rescue of ?F508-CFTR Mimics Genetic Repair in Cystic Fibrosis Bronchial Epithelial, Cells Mol Cell Proteomics, vol.7, issue.6, pp.1099-1110, 2008. ,
Proteomic Analysis of Nasal Epithelial Cells from Cystic Fibrosis Patients, PLoSONE, vol.9, issue.9, 2014. ,
URL : https://hal.archives-ouvertes.fr/hal-01361837
?F508 CFTR interactome remodelling promotes rescue of cystic fibrosis, Nature, vol.528, issue.7583, pp.510-516, 2015. ,
Expression of CCT6A mRNA in chicken granulosa cells is regulated by progesterone, General and Comparative Endocrinology, vol.189, pp.15-23, 2013. ,
In vivo newly translated polypeptides are sequestered in a protected folding environment, EMBO J, vol.18, pp.85-95, 1999. ,
The t-complex polypeptide 1 complex is a chaperonin for tubulin and actin in vivo, Proc. Natl. Acad. Sci, vol.90, pp.9422-9426, 1993. ,
Agonist-induced internalization and downregulation of gonadotropin-releasing hormone receptors, Am J Physiol Cell Physiol, vol.297, issue.3, pp.591-600, 2009. ,
Gonadotropin-Releasing Hormone and Its Physiological and Pathophysiological Roles in Relation to the Structure and Function of the Gastrointestinal Tract, European surgical Research, vol.57, pp.1-2, 2016. ,
Short-term buserelin administration induces apoptosis and morphological changes in adult rat testes, Acta Cir. Bras, vol.32, pp.140-147, 2017. ,
Efficacy of intranasal or subcutaneous luteinizing hormone-releasing hormone agonist inhibition of ovarian function in the treatment of endometriosis, Am J Obstet Gynecol, vol.158, issue.2, pp.233-239, 1988. ,