B. Kerem, J. Rommens, J. Buchanan, D. Markiewicz, T. Cox et al., Identification of the cystic fibrosis gene: genetic analysis, Science, vol.245, issue.4922, pp.1073-1080, 1989.
DOI : 10.1126/science.2570460

B. Quon and M. Aitken, Cystic Fibrosis: What to Expect now in the Early Adult Years, Paediatric Respiratory Reviews, vol.13, issue.4, pp.206-214, 2012.
DOI : 10.1016/j.prrv.2012.03.005

B. Ramsey, S. Banks-schlegel, F. Accurso, R. Boucher, G. Cutting et al., Future Directions in Early Cystic Fibrosis Lung Disease Research, American Journal of Respiratory and Critical Care Medicine, vol.185, issue.8
DOI : 10.1164/rccm.201111-2068WS

C. Goss and F. Ratjen, Update in Cystic Fibrosis 2012, American Journal of Respiratory and Critical Care Medicine, vol.187, issue.9, pp.915-919, 2013.
DOI : 10.1164/rccm.201301-0184UP

R. Aris, P. Merkel, L. Bachrach, D. Borowitz, M. Boyle et al., 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

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

I. Sermet-gaudelus, J. Souberbielle, J. Ruiz, S. Vrielynck, B. Heuillon et al., 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

A. Sparks, S. Mcgee, C. Boone, I. Neuringer, S. Jones et al., ???Old??? bones in young bodies: the tale of cystic fibrosis, Current Opinion in Endocrinology, Diabetes and Obesity, vol.16, issue.6, pp.407-414, 2009.
DOI : 10.1097/MED.0b013e3283329b16

E. Shane, S. Silverberg, D. Donovan, A. Papadopoulos, R. Staron et al., Osteoporosis in lung transplantation candidates with end-stage pulmonary disease, The American Journal of Medicine, vol.101, issue.3, pp.262-269, 1996.
DOI : 10.1016/S0002-9343(96)00155-6

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. Rossini, D. Marco, A. , D. Santo, F. Gatti et al., Prevalence and correlates of vertebral fractures in adults with cystic fibrosis, Bone, vol.35, issue.3, pp.771-776, 2004.
DOI : 10.1016/j.bone.2004.05.009

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

E. Shead, C. Haworth, E. Gunn, D. Bilton, M. Scott et al., 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. Haston, W. Li, A. Li, M. Lafleur, and J. Henderson, 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

M. Stalvey, K. Clines, V. Havasi, C. Mckibbin, L. Dunn et al., 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

L. Henaff, C. Gimenez, A. Hay, E. Marty, C. Marie et al., 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

J. Paradis, M. Wilke, and C. Haston, Osteopenia in Cftr-deltaF508 mice, Journal of Cystic Fibrosis, vol.9, issue.4, pp.239-245, 2011.
DOI : 10.1016/j.jcf.2010.05.001

E. Shead, C. Haworth, A. Condliffe, D. Mckeon, M. Scott et al., 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

A. Gimenez, L. Henaff, C. Norez, C. Guillaume, C. Ravoninjatovo et al., Deficit of osteoprotegerin release by osteoblasts from a patient with cystic fibrosis, European Respiratory Journal, vol.39, issue.3, pp.780-781, 2012.
DOI : 10.1183/09031936.00104111

URL : https://hal.archives-ouvertes.fr/inserm-00602058

D. Galanaud, A. Tourbah, S. Lehericy, N. Leveque, B. Heron et al., 24 month-treatment with miglustat of three patients with Niemann-Pick disease type C: Follow up using brain spectroscopy, Molecular Genetics and Metabolism, vol.96, issue.2, pp.55-58, 2009.
DOI : 10.1016/j.ymgme.2008.10.002

URL : https://hal.archives-ouvertes.fr/hal-00805433

G. Pastores, D. Elstein, M. Hrebicek, and A. Zimran, Effect of Miglustat on Bone Disease in Adults with Type 1 Gaucher Disease: A Pooled Analysis of Three Multinational, Open-Label Studies, Clinical Therapeutics, vol.29, issue.8, pp.1645-1654, 2007.
DOI : 10.1016/j.clinthera.2007.08.006

C. Norez, F. Antigny, S. Noel, C. Vandebrouck, and F. Becq, A Cystic Fibrosis Respiratory Epithelial Cell Chronically Treated by Miglustat Acquires a Non???Cystic Fibrosis???Like Phenotype, American Journal of Respiratory Cell and Molecular Biology, vol.41, issue.2, pp.217-225, 2009.
DOI : 10.1165/rcmb.2008-0285OC

URL : https://hal.archives-ouvertes.fr/hal-00444213

M. Egan, M. Pearson, S. Weiner, V. Rajendran, D. Rubin et al., Curcumin, a Major Constituent of Turmeric, Corrects Cystic Fibrosis Defects, Science, vol.304, issue.5670, pp.600-602, 2004.
DOI : 10.1126/science.1093941

C. Norez, S. Noel, M. Wilke, M. Bijvelds, H. Jorna et al., 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 : 10.1016/j.febslet.2006.03.010

B. Lubamba, J. Lebacq, P. Lebecque, R. Vanbever, A. Leonard et al., Airway Delivery of Low-Dose Miglustat Normalizes Nasal Potential Difference in F508del Cystic Fibrosis Mice, American Journal of Respiratory and Critical Care Medicine, vol.179, issue.11, pp.1022-1028, 2009.
DOI : 10.1164/rccm.200901-0049OC

A. Leonard, P. Lebecque, J. Dingemanse, and T. Leal, A randomized placebo-controlled trial of miglustat in cystic fibrosis based on nasal potential difference, Journal of Cystic Fibrosis, vol.11, issue.3, pp.231-236, 2012.
DOI : 10.1016/j.jcf.2011.12.004

B. Jenkins and L. Glenn, Miglustat effects on the basal nasal potential differences in cystic fibrosis, Journal of Cystic Fibrosis, vol.12, issue.1, p.88, 2012.
DOI : 10.1016/j.jcf.2012.06.003

J. Van-doorninck, P. French, E. Verbeek, R. Peters, H. Morreau et al., A mouse model for the cystic fibrosis delta F508 mutation, Embo J, vol.14, pp.4403-4411, 1995.

C. Cottart, E. Bonvin, C. Rey, D. Wendum, J. Bernaudin et al., Impact of Nutrition on Phenotype in CFTR-deficient Mice, Pediatric Research, vol.2, issue.5, pp.528-532, 2007.
DOI : 10.1203/PDR.0b013e318155a61d

S. Dorozhkin and M. Epple, Biological and Medical Significance of Calcium Phosphates, Angewandte Chemie International Edition, vol.106, issue.311, pp.3130-3146, 2002.
DOI : 10.1002/1521-3773(20020902)41:17<3130::AID-ANIE3130>3.0.CO;2-1

A. Parfitt, M. Drezner, F. Glorieux, J. Kanis, H. Malluche et al., Bone histomorphometry: Standardization of nomenclature, symbols, and units: Report of the asbmr histomorphometry nomenclature committee, Journal of Bone and Mineral Research, vol.10, issue.6, pp.595-610, 1987.
DOI : 10.1002/jbmr.5650020617

V. Saint-criq, C. Rebeyrol, M. Ruffin, T. Roque, L. Guillot et al., Restoration of Chloride Efflux by Azithromycin in Airway Epithelial Cells of Cystic Fibrosis Patients, Antimicrobial Agents and Chemotherapy, vol.55, issue.4, pp.1792-1793, 2012.
DOI : 10.1128/AAC.01403-10

C. Bonifant, E. Shevill, and A. Chang, Vitamin A supplementation for cystic fibrosis, Cochrane Database Syst Rev, vol.8, p.6751, 2012.

L. Hillman, J. Cassidy, M. Popescu, J. Hewett, J. Kyger et al., Percent true calcium absorption, mineral metabolism, and bone mineralization in children with cystic fibrosis: Effect of supplementation with vitamin D and calcium, Pediatric Pulmonology, vol.88, issue.8, pp.772-780, 2008.
DOI : 10.1002/ppul.20863

A. Giustina, G. Mazziotti, and E. Canalis, Growth Hormone, Insulin-Like Growth Factors, and the Skeleton, Endocrine Reviews, vol.29, issue.5, pp.535-559, 2008.
DOI : 10.1210/er.2007-0036

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

F. Festini, G. Taccetti, T. Repetto, M. Reali, S. Campana et al., Gestational and Neonatal Characteristics of Children with Cystic Fibrosis: A Cohort Study, The Journal of Pediatrics, vol.147, issue.3, pp.316-320, 2005.
DOI : 10.1016/j.jpeds.2005.04.031

M. Switzer, J. Rice, M. Rice, and D. Hardin, Insulin-like Growth Factor-I Levels Predict Weight, Height and Protein Catabolism in Children and Adolescents with Cystic Fibrosis, Journal of Pediatric Endocrinology and Metabolism, vol.22, issue.5, pp.417-424, 2009.
DOI : 10.1515/JPEM.2009.22.5.417

M. Rogan, L. Reznikov, A. Pezzulo, N. Gansemer, M. Samuel et al., Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth, Proceedings of the National Academy of Sciences, vol.107, issue.47, pp.20571-20575, 2000.
DOI : 10.1073/pnas.1015281107

S. Elkin, S. Vedi, S. Bord, N. Garrahan, M. Hodson et al., Histomorphometric Analysis of Bone Biopsies from the Iliac Crest of Adults with Cystic Fibrosis, American Journal of Respiratory and Critical Care Medicine, vol.166, issue.11, pp.1470-1474, 1999.
DOI : 10.1164/rccm.200206-578OC

S. King, D. Topliss, T. Kotsimbos, I. Nyulasi, M. Bailey et al., Reduced bone density in cystic fibrosis: ??F508 mutation is an independent risk factor, European Respiratory Journal, vol.25, issue.1, pp.54-61, 2005.
DOI : 10.1183/09031936.04.00050204

Y. Imai, S. Kondoh, A. Kouzmenko, and S. Kato, Minireview: Osteoprotective Action of Estrogens Is Mediated by Osteoclastic Estrogen Receptor-??, Molecular Endocrinology, vol.24, issue.5, pp.877-885, 2011.
DOI : 10.1210/me.2009-0238

H. Chen, J. Guo, Y. Lu, G. Ding, M. Yu et al., Impaired CFTR-Dependent Amplification of FSH-Stimulated Estrogen Production in Cystic Fibrosis and PCOS, The Journal of Clinical Endocrinology & Metabolism, vol.97, issue.3, pp.923-932, 2012.
DOI : 10.1210/jc.2011-1363

T. Fanelli, R. Cardone, M. Favia, L. Guerra, M. Zaccolo et al., ??-Oestradiol rescues ??F508CFTR functional expression in human cystic fibrosis airway CFBE41o??? cells through the up-regulation of NHERF1, Biology of the Cell, vol.282, issue.7, pp.399-412, 2008.
DOI : 10.1042/BC20070095

S. Noel, M. Wilke, A. Bot, D. Jonge, H. Becq et al., Parallel Improvement of Sodium and Chloride Transport Defects by Miglustat (n-Butyldeoxynojyrimicin) in Cystic Fibrosis Epithelial Cells, Journal of Pharmacology and Experimental Therapeutics, vol.325, issue.3, pp.1016-1023, 2008.
DOI : 10.1124/jpet.107.135582

URL : https://hal.archives-ouvertes.fr/hal-00402085

F. Platt, G. Neises, G. Karlsson, R. Dwek, and T. Butters, N-butyldeoxygalactonojirimycin inhibits glycolipid biosynthesis but does not affect N-linked oligosaccharide processing, J Biol Chem, vol.269, pp.27108-27114, 1994.

E. Gronowitz, D. Mellstrom, and B. Strandvik, Serum phospholipid fatty acid pattern is associated with bone mineral density in children, but not adults, with cystic fibrosis, British Journal of Nutrition, vol.95, issue.06, pp.1159-1165, 2006.
DOI : 10.1079/BJN20061778

C. Guilbault, D. Sanctis, J. Wojewodka, G. Saeed, Z. Lachance et al., Fenretinide Corrects Newly Found Ceramide Deficiency in Cystic Fibrosis, American Journal of Respiratory Cell and Molecular Biology, vol.38, issue.1, pp.47-56, 2008.
DOI : 10.1165/rcmb.2007-0036OC

R. Aris, J. Renner, A. Winders, H. Buell, D. Riggs et al., 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

J. Paccou, N. Zeboulon, C. Combescure, L. Gossec, and B. Cortet, 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

M. Whyte, W. Mcalister, D. Novack, K. Clements, P. Schoenecker et al., Bisphosphonate-Induced Osteopetrosis: Novel Bone Modeling Defects, Metaphyseal Osteopenia, and Osteosclerosis Fractures After Drug Exposure Ceases, Journal of Bone and Mineral Research, vol.92, issue.S1, pp.1698-1707, 2008.
DOI : 10.1359/jbmr.080511

A. Treiber, O. Morand, and M. Clozel, The pharmacokinetics and tissue distribution of the glucosylceramide synthase inhibitor miglustat in the rat, Xenobiotica, vol.358, issue.3, pp.298-314, 2007.
DOI : 10.1016/S0140-6736(00)02161-9

R. Venier and S. Igdoura, Miglustat as a therapeutic agent: prospects and caveats, Journal of Medical Genetics, vol.49, issue.9, pp.591-597, 2012.
DOI : 10.1136/jmedgenet-2012-101070