J. Riordan, J. Rommens, and B. Kerem, Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA [erratum in Science, Science, vol.245, issue.245, pp.1437-1066, 1989.

D. Stoltz, D. Meyerholz, and M. Welsh, Origins of cystic fibrosis lung disease, N Engl J Med, vol.372, pp.351-362, 2015.

M. Boyle, D. Boeck, and K. , A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect, The Lancet Respiratory Medicine, vol.1, issue.2, pp.158-163, 2013.
DOI : 10.1016/S2213-2600(12)70057-7

J. Riordan, 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

J. Jacquot, M. Delion, and S. Gangloff, Bone disease in cystic fibrosis: new pathogenic insights opening novel therapies, Osteoporosis International, vol.365, issue.Suppl 2, pp.1401-1412, 2016.
DOI : 10.1007/s00198-015-3343-3

URL : https://hal.archives-ouvertes.fr/inserm-01221835

B. Plant, C. Goss, and W. Plant, Management of comorbidities in older patients with cystic fibrosis, The Lancet Respiratory Medicine, vol.1, issue.2, pp.164-174, 2013.
DOI : 10.1016/S2213-2600(13)70025-0

M. Putman, C. Milliren, and N. Derrico, Compromised Bone Microarchitecture and Estimated Bone Strength in Young Adults With Cystic Fibrosis, The Journal of Clinical Endocrinology & Metabolism, vol.99, issue.9, pp.3399-3407, 2014.
DOI : 10.1210/jc.2014-1982

R. Aris, P. Merkel, and L. Bachrach, Guide to Bone Health and Disease in Cystic Fibrosis, The Journal of Clinical Endocrinology & Metabolism, vol.90, issue.3, pp.1888-1896, 2005.
DOI : 10.1210/jc.2004-1629

H. Buntain, R. Greer, and P. Schluter, Bone mineral density in Australian children, adolescents and adults with cystic fibrosis: a controlled cross sectional study, Thorax, vol.59, issue.2, pp.149-155, 2004.
DOI : 10.1136/thorax.2003.006726

J. Paccou, N. Zeboulon, and C. Combescure, The Prevalence of Osteoporosis, Osteopenia, and Fractures Among Adults with Cystic Fibrosis: A Systematic Literature Review with Meta-Analysis, Calcified Tissue International, vol.134, issue.1, pp.1-7, 2010.
DOI : 10.1007/s00223-009-9316-9

I. Sermet-gaudelus, M. Castanet, and G. Retsch-bogart, Update on Cystic Fibrosis-Related Bone Disease: A Special Focus on Children, Paediatric Respiratory Reviews, vol.10, issue.3, pp.134-142, 2009.
DOI : 10.1016/j.prrv.2009.05.001

R. Aris, J. Renner, and A. Winders, Increased Rate of Fractures and Severe Kyphosis: Sequelae of Living into Adulthood with Cystic Fibrosis, Annals of Internal Medicine, vol.128, issue.3, pp.186-193, 1998.
DOI : 10.7326/0003-4819-128-3-199802010-00004

F. Velard, M. Delion, L. Henaff, and C. , Cystic Fibrosis and Bone Disease: Defective Osteoblast Maturation with the F508del Mutation in Cystic Fibrosis Transmembrane Conductance Regulator, American Journal of Respiratory and Critical Care Medicine, vol.189, issue.6, pp.746-748, 2014.
DOI : 10.1164/rccm.201312-2144LE

URL : https://hal.archives-ouvertes.fr/inserm-00965536

A. Gore, S. Kwon, and A. Stenbit, A Roadmap to the Brittle Bones of Cystic Fibrosis, Journal of Osteoporosis, vol.11, issue.1, p.926045, 2010.
DOI : 10.1038/ncprheum0977

M. Boyle, Update on maintaining bone health in cystic fibrosis, Current Opinion in Pulmonary Medicine, vol.12, issue.6, pp.453-458, 2006.
DOI : 10.1097/01.mcp.0000245708.59138.a4

C. Haworth, Impact of cystic fibrosis on bone health, Current Opinion in Pulmonary Medicine, vol.16, issue.6, pp.616-622, 2010.
DOI : 10.1097/MCP.0b013e32833e2e94

R. Javier and J. Jacquot, Bone disease in cystic fibrosis: What's new?, Joint Bone Spine, vol.78, issue.5, pp.445-450, 2011.
DOI : 10.1016/j.jbspin.2010.11.015

M. Stalvey and G. Clines, Cystic fibrosis-related bone disease, Current Opinion in Endocrinology & Diabetes and Obesity, vol.20, issue.6, pp.547-552, 2013.
DOI : 10.1097/01.med.0000436191.87727.ec

M. Putman, L. Greenblatt, and L. Sicilian, Young adults with cystic fibrosis have altered trabecular microstructure by ITS-based morphological analysis, Osteoporosis International, vol.100, issue.1, pp.10-1007, 2016.
DOI : 10.1007/s00198-015-3343-3

I. Sermet-gaudelus, J. Souberbielle, and J. Ruiz, Low Bone Mineral Density in Young Children with Cystic Fibrosis, American Journal of Respiratory and Critical Care Medicine, vol.175, issue.9, pp.951-957, 2007.
DOI : 10.1164/rccm.200606-776OC

L. Heron, L. Guillaume, C. Velard, and F. , Cystic fibrosis transmembrane conductance regulator (CFTR) regulates the production of osteoprotegerin (OPG) and prostaglandin (PG) E2 in human bone, Journal of Cystic Fibrosis, vol.9, issue.1, pp.69-72, 2010.
DOI : 10.1016/j.jcf.2009.11.005

URL : https://hal.archives-ouvertes.fr/inserm-00501818

E. Shead, C. Haworth, and A. Condliffe, Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human bone, Thorax, vol.62, issue.7, pp.650-651, 2007.
DOI : 10.1136/thx.2006.075887

L. Henaff, C. Gimenez, A. Hay, and E. , The F508del Mutation in Cystic Fibrosis Transmembrane Conductance Regulator Gene Impacts Bone Formation, The American Journal of Pathology, vol.180, issue.5, pp.2068-2075, 2012.
DOI : 10.1016/j.ajpath.2012.01.039

URL : https://hal.archives-ouvertes.fr/inserm-00846637

L. Henaff, C. Hay, E. Velard, and F. , Enhanced F508del-CFTR Channel Activity Ameliorates Bone Pathology in Murine Cystic Fibrosis, The American Journal of Pathology, vol.184, issue.4, pp.1132-1141, 2014.
DOI : 10.1016/j.ajpath.2013.12.027

URL : https://hal.archives-ouvertes.fr/inserm-00994801

R. Baron and M. Kneissel, WNT signaling in bone homeostasis and disease: from human mutations to treatments, Nature Medicine, vol.28, issue.2, pp.179-192, 2013.
DOI : 10.1016/j.bone.2009.02.012

S. Khosla, Minireview: The OPG/RANKL/RANK System, Endocrinology, vol.142, issue.12, pp.5050-5055, 2001.
DOI : 10.1210/endo.142.12.8536

S. Teitelbaum, Osteoclasts: What Do They Do and How Do They Do It?, The American Journal of Pathology, vol.170, issue.2, pp.427-435, 2007.
DOI : 10.2353/ajpath.2007.060834

S. Wei and G. Siegal, Mechanisms modulating inflammatory osteolysis: A review with insights into therapeutic targets, Pathology - Research and Practice, vol.204, issue.10, pp.695-706, 2008.
DOI : 10.1016/j.prp.2008.07.002

B. Boyce and L. Xing, Functions of RANKL/RANK/OPG in bone modeling and remodeling, Archives of Biochemistry and Biophysics, vol.473, issue.2, pp.139-146, 2008.
DOI : 10.1016/j.abb.2008.03.018

D. Lacey, E. Timms, and H. Tan, Osteoprotegerin Ligand Is a Cytokine that Regulates Osteoclast Differentiation and Activation, Cell, vol.93, issue.2, pp.165-176, 1998.
DOI : 10.1016/S0092-8674(00)81569-X

E. Shead, C. Haworth, and H. Barker, Osteoclast function, bone turnover and inflammatory cytokines during infective exacerbations of cystic fibrosis, Journal of Cystic Fibrosis, vol.9, issue.2, pp.93-98, 2010.
DOI : 10.1016/j.jcf.2009.11.007

J. Ambroszkiewicz, D. Sands, and J. Gajewska, Bone turnover markers, osteoprotegerin and RANKL cytokines in children with cystic fibrosis, Advances in Medical Sciences, vol.58, issue.2, pp.338-343, 2013.
DOI : 10.2478/ams-2013-0011

K. Blackwell, L. Raisz, and C. Pilbeam, Prostaglandins in bone: bad cop, good cop?, Trends in Endocrinology & Metabolism, vol.21, issue.5, pp.294-301, 2010.
DOI : 10.1016/j.tem.2009.12.004

C. Xie, B. Liang, and M. Xue, Rescue of Impaired Fracture Healing in COX-2???/??? Mice via Activation of Prostaglandin E2 Receptor Subtype 4, The American Journal of Pathology, vol.175, issue.2, pp.772-785, 2009.
DOI : 10.2353/ajpath.2009.081099

K. Kim, H. Kim, and S. Lee, 15-Deoxy-? 12,14 -prostaglandin J 2 inhibits osteolytic breast cancer bone metastasis and estrogen deficiency-induced bone loss, PLoS One, vol.10, p.122764, 2015.

M. Napimoga, A. Demasi, and J. Bossonaro, Low doses of 15d-PGJ2 induce osteoblast activity in a PPAR-gamma independent manner, International Immunopharmacology, vol.16, issue.2, pp.131-138, 2013.
DOI : 10.1016/j.intimp.2013.03.035

X. Zhang, E. Schwarz, and D. Young, Cyclooxygenase-2 regulates mesenchymal cell differentiation into the osteoblast lineage and is critically involved in bone repair, Journal of Clinical Investigation, vol.109, issue.11, pp.1405-1415, 2002.
DOI : 10.1172/JCI0215681

A. Tyagi, K. Srivastava, and M. Mansoori, Estrogen Deficiency Induces the Differentiation of IL-17 Secreting Th17 Cells: A New Candidate in the Pathogenesis of Osteoporosis, PLoS ONE, vol.7, issue.9, p.44552, 2012.
DOI : 10.1371/journal.pone.0044552.s001

X. Liu, A. Kirschenbaum, and S. Yao, Interactive Effect of Interleukin-6 and Prostaglandin E2 on Osteoclastogenesis via the OPG/RANKL/RANK System, Annals of the New York Academy of Sciences, vol.1068, issue.1, pp.225-233, 2006.
DOI : 10.1172/JCI200419872

M. Stalvey, K. Clines, and V. Havasi, Osteoblast CFTR Inactivation Reduces Differentiation and Osteoprotegerin Expression in a Mouse Model of Cystic Fibrosis-Related Bone Disease, PLoS ONE, vol.349, issue.11, p.80098, 2013.
DOI : 10.1371/journal.pone.0080098.g005

K. Tiringer, A. Treis, and P. Fucik, Infection, American Journal of Respiratory and Critical Care Medicine, vol.187, issue.6, pp.621-629, 2013.
DOI : 10.1164/rccm.201206-1150OC

S. Kotake, N. Udagawa, and N. Takahashi, IL-17 in synovial fluids from patients with rheumatoid arthritis is a potent stimulator of osteoclastogenesis, Journal of Clinical Investigation, vol.103, issue.9, pp.1345-1352, 1999.
DOI : 10.1172/JCI5703

G. Mori, D. Amelio, P. Faccio, and R. , Bone-Immune Cell Crosstalk: Bone Diseases, Journal of Immunology Research, vol.2, issue.1, p.108451, 2015.
DOI : 10.1158/0008-5472.CAN-12-2202

URL : http://doi.org/10.1155/2015/108451

A. Verkman and S. Jayaraman, Fluorescent Indicator Methods to Assay Functional CFTR Expression in Cells, Methods Mol Med, vol.70, pp.187-196, 2002.
DOI : 10.1385/1-59259-187-6:187

F. Van-goor, S. Hadida, and P. Grootenhuis, Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770, Proceedings of the National Academy of Sciences, vol.106, issue.44, pp.18825-18830, 2009.
DOI : 10.1073/pnas.0904709106

P. Eckford, M. Ramjeesingh, and S. Molinski, VX-809 and Related Corrector Compounds Exhibit Secondary Activity Stabilizing Active F508del-CFTR after Its Partial Rescue to the Cell Surface, Chemistry & Biology, vol.21, issue.5, pp.666-678, 2014.
DOI : 10.1016/j.chembiol.2014.02.021

S. Bitam, I. Pranke, and M. Hollenhorst, An unexpected effect of TNF-alpha on F508del-CFTR maturation and function, F1000Res, vol.4, p.218, 2015.

M. Baudouin-legros, J. Colas, and S. Moriceau, Long-term CFTR inhibition modulates 15d-prostaglandin J2 in human pulmonary cells, The International Journal of Biochemistry & Cell Biology, vol.44, issue.6
DOI : 10.1016/j.biocel.2012.03.010

F. Velard, M. Delion, and F. Lemaire, Cystic fibrosis bone disease: is the CFTR corrector C18 an option for therapy?, European Respiratory Journal, vol.45, issue.3, pp.845-848, 2015.
DOI : 10.1183/09031936.00174014

F. Dif, C. Marty, and C. Baudoin, Severe osteopenia in CFTR-null mice, Bone, vol.35, issue.3, pp.595-603, 2004.
DOI : 10.1016/j.bone.2004.05.021

URL : https://hal.archives-ouvertes.fr/hal-00181103

C. Haston, W. Li, and A. Li, Persistent Osteopenia in Adult Cystic Fibrosis Transmembrane Conductance Regulator???deficient Mice, American Journal of Respiratory and Critical Care Medicine, vol.177, issue.3, pp.309-315, 2008.
DOI : 10.1164/rccm.200705-659OC

T. Pashuck, S. Franz, and M. Altman, Murine Model for Cystic Fibrosis Bone Disease Demonstrates Osteopenia and Sex-Related Differences in Bone Formation, Pediatric Research, vol.7, issue.3, pp.311-316, 2009.
DOI : 10.1203/PDR.0b013e3181961e80

I. Sermet-gaudelus, M. Bianchi, and M. Garabedian, European cystic fibrosis bone mineralisation guidelines, Journal of Cystic Fibrosis, vol.10, issue.2, pp.16-23, 2011.
DOI : 10.1016/S1569-1993(11)60004-0

J. Ousingsawat, P. Wanitchakool, and R. Schreiber, Anoctamin-6 Controls Bone Mineralization by Activating the Calcium Transporter NCX1, Journal of Biological Chemistry, vol.290, issue.10, pp.6270-6280, 2015.
DOI : 10.1074/jbc.M114.602979

E. Shead, C. Haworth, and E. Gunn, Osteoclastogenesis during Infective Exacerbations in Patients with Cystic Fibrosis, American Journal of Respiratory and Critical Care Medicine, vol.174, issue.3, pp.306-311, 2006.
DOI : 10.1164/rccm.200512-1943OC

C. Haworth, P. Selby, and A. Webb, Inflammatory related changes in bone mineral content in adults with cystic fibrosis, Thorax, vol.59, issue.7, pp.613-617, 2004.
DOI : 10.1136/thx.2003.012047

G. Shmarina, A. Pukhalsky, and N. Petrova, TNF gene polymorphisms in cystic fibrosis patients: contribution to the disease progression, Journal of Translational Medicine, vol.11, issue.1, p.19, 2013.
DOI : 10.1016/j.jcf.2011.09.006

S. Krum, J. Chang, and G. Miranda-carboni, Novel functions for NF??B: inhibition of bone formation, Nature Reviews Rheumatology, vol.276, issue.10, pp.607-611, 2011.
DOI : 10.1038/nrrheum.2010.133

J. Chang, Z. Wang, and E. Tang, Inhibition of osteoblastic bone formation by nuclear factor-??B, Nature Medicine, vol.12, issue.6, pp.682-689, 2009.
DOI : 10.1038/nm.1954

E. Dimango, A. Ratner, and R. Bryan, Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells., Journal of Clinical Investigation, vol.101, issue.11, pp.2598-2605, 1998.
DOI : 10.1172/JCI2865

O. Tabary, S. Escotte, and J. Couetil, Genistein Inhibits Constitutive and Inducible NF??B Activation and Decreases IL-8 Production by Human Cystic Fibrosis Bronchial Gland Cells, The American Journal of Pathology, vol.155, issue.2, pp.473-481, 1999.
DOI : 10.1016/S0002-9440(10)65143-7

N. Vij, S. Mazur, and P. Zeitlin, CFTR Is a Negative Regulator of NF??B Mediated Innate Immune Response, PLoS ONE, vol.31, issue.2, p.4664, 2009.
DOI : 10.1371/journal.pone.0004664.s004

D. Straus, G. Pascual, and M. Li, 15-Deoxy-Delta 12,14-prostaglandin J2 inhibits multiple steps in the NF-kappa B signaling pathway, Proceedings of the National Academy of Sciences, vol.97, issue.9, pp.4844-4849, 2000.
DOI : 10.1073/pnas.97.9.4844

L. Conwell and A. Chang, Bisphosphonates for osteoporosis in people with cystic fibrosis, Cochrane Database Syst Rev, vol.3, p.2010, 2014.

D. Lacey, W. Boyle, and W. Simonet, Bench to bedside: elucidation of the OPG???RANK???RANKL pathway and the development of denosumab, Nature Reviews Drug Discovery, vol.46, issue.5, pp.401-419, 2012.
DOI : 10.1038/nrd3705

S. Cohen, R. Dore, and N. Lane, Denosumab treatment effects on structural damage, bone mineral density, and bone turnover in rheumatoid arthritis: A twelve-month, multicenter, randomized, double-blind, placebo-controlled, phase II clinical trial, Arthritis & Rheumatism, vol.27, issue.5, pp.1299-1309, 2008.
DOI : 10.1002/art.23417

V. Birault, R. Solari, and J. Hanrahan, Correctors of the basic trafficking defect of the mutant F508del-CFTR that causes cystic fibrosis, Current Opinion in Chemical Biology, vol.17, issue.3, pp.353-360, 2013.
DOI : 10.1016/j.cbpa.2013.04.020