M. Gao and . Goldfarb, Receptor specificity of the fibroblast growth factor family, J. Biol

M. L. Maroteaux, A. Merrer, and . Munnich, Mutations in the gene encoding fibroblast growth factor receptor-3 in achondroplasia, Nature, vol.371, pp.252-254, 1994.

G. A. Bellus, I. Mcintosh, E. A. Smith, A. S. Aylsworth, I. Kaitila et al., A recurrent mutation in the tyrosine kinase domain of fibroblast growth factor receptor 3 causes hypochondroplasia, Nature Genetics, vol.6, issue.3
DOI : 10.1038/ng0295-165

J. Maroteaux, F. Bonaventure, M. Narcy, and . Sanak, Stop codon FGFR3 mutations in thanatophoric dwarfism type 1, Nat. Genet, vol.10, pp.11-12, 1995.

J. Munnich and . Bonaventure, Missense FGFR3 mutations create cysteine residues in thanatophoric dwarfism type I (TD1), Hum. Mol. Genet, vol.5, pp.509-512, 1996.

M. C. Naski, Q. Wang, J. Xu, and D. M. Ornitz, Graded activation of fibroblast growth factor receptor 3 by mutations causing achondroplasia and thanatophoric dysplasia, Nature Genetics, vol.84, issue.2, pp.13-233, 1996.
DOI : 10.1038/ng0696-233

M. C. Naski, J. S. Colvin, J. D. Coffin, and D. M. Ornitz, Repression of hedgehog signaling and BMP4 expression in growth plate cartilage by fibroblast growth factor receptor 3, Development, vol.125, pp.4977-4988, 1998.

L. Chen, R. Adar, X. Yang, E. O. Monsonego, C. Li et al., Gly369Cys mutation in mouse FGFR3 causes achondroplasia by affecting both chondrogenesis and osteogenesis, Journal of Clinical Investigation, vol.104, issue.11, pp.1517-1525, 1999.
DOI : 10.1172/JCI6690

Y. Wang, M. K. Spatz, K. Kannan, H. Hayk, A. Avivi et al., A mouse model for achondroplasia produced by targeting fibroblast growth factor receptor 3, Proceedings of the National Academy of Sciences, vol.96, issue.8, pp.96-4455, 1999.
DOI : 10.1073/pnas.96.8.4455

T. Iwata, L. Chen, C. Li, D. A. Ovchinnikov, R. R. Behringer et al., A neonatal lethal mutation in FGFR3 uncouples proliferation and differentiation of growth plate chondrocytes in embryos, Human Molecular Genetics, vol.9, issue.11, pp.1603-1613, 2000.
DOI : 10.1093/hmg/9.11.1603

T. Iwata, C. L. Li, C. X. Deng, and C. A. Francomano, Highly activated Fgfr3 with the K644M mutation causes prolonged survival in severe dwarf mice, Human Molecular Genetics, vol.10, issue.12
DOI : 10.1093/hmg/10.12.1255

C. Li, L. Chen, T. Iwata, M. Kitagawa, X. Y. Fu et al., A Lys644Glu substitution in fibroblast growth factor receptor 3 (FGFR3) causes dwarfism in mice by activation of STATs and ink4 cell cycle inhibitors, Human Molecular Genetics, vol.8, issue.1, pp.35-44, 1999.
DOI : 10.1093/hmg/8.1.35

J. S. Colvin, B. A. Bohne, G. W. Harding, D. G. Mcewen, and D. M. Ornitz, Skeletal overgrowth and deafness in mice lacking fibroblast growth factor receptor 3, Nature Genetics, vol.25, issue.4
DOI : 10.1016/0092-8674(91)90034-V

K. L. Mueller, B. E. Jacques, and M. W. Kelley, Fibroblast growth factor signaling regulates pillar cell development in the organ of corti, J. Neurosci, vol.22, pp.9368-9377, 2002.

T. Hayashi, D. Cunningham, and O. Bermingham-mcdonogh, Loss of Fgfr3 leads to excess hair cell development in the mouse organ of Corti, Developmental Dynamics, vol.21, issue.2, pp.525-533, 2007.
DOI : 10.1002/dvdy.21026

M. W. Fritzsch and . Kelley, Disruption of fibroblast growth factor receptor 3 signaling results in defects in cellular differentiation, neuronal patterning, and hearing impairment, Dev. Dyn, vol.236, pp.1905-1917, 2007.

M. W. Kelley, Regulation of cell fate in the sensory epithelia of the inner ear

L. Gibbs and L. , FGFR3 intracellular mutations induce tyrosine phosphorylation in the Golgi and defective glycosylation, Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, vol.1773, issue.4, pp.502-512, 2007.
DOI : 10.1016/j.bbamcr.2006.12.010

D. Metzger, J. Clifford, H. Chiba, and P. Chambon, Conditional site-specific recombination in mammalian cells using a ligand-dependent chimeric Cre recombinase., Proceedings of the National Academy of Sciences, vol.92, issue.15, pp.92-6991, 1995.
DOI : 10.1073/pnas.92.15.6991

C. Deng, A. Wynshaw-boris, F. Zhou, A. Kuo, and P. Leder, Fibroblast Growth Factor Receptor 3 Is a Negative Regulator of Bone Growth, Cell, vol.84, issue.6, pp.911-921, 1996.
DOI : 10.1016/S0092-8674(00)81069-7

A. M. Ouagazzal, D. Reiss, R. Romand, and R. , Effects of age-related hearing loss on startle reflex and prepulse inhibition in mice on pure and mixed C57BL and 129 genetic background, Behavioural Brain Research, vol.172, issue.2, pp.172-307, 2006.
DOI : 10.1016/j.bbr.2006.05.018

URL : https://hal.archives-ouvertes.fr/hal-00188095

B. R. Olsen, A. M. Reginato, and W. Wang, Bone Development, Annual Review of Cell and Developmental Biology, vol.16, issue.1, pp.191-220, 2000.
DOI : 10.1146/annurev.cellbio.16.1.191

URL : https://hal.archives-ouvertes.fr/hal-00306959

L. Chen, C. Li, W. Qiao, X. Xu, and C. Deng, A Ser365->Cys mutation of fibroblast growth factor receptor 3 in mouse downregulates Ihh/PTHrP signals and causes severe achondroplasia, Human Molecular Genetics, vol.10, issue.5, pp.457-465, 2001.
DOI : 10.1093/hmg/10.5.457

P. Dallos, The active cochlea, J. Neurosci, vol.12, pp.4575-4585, 1992.

K. Shim, G. Minowada, D. E. Coling, and G. R. Martin, Sprouty2, a Mouse Deafness Gene, Regulates Cell Fate Decisions in the Auditory Sensory Epithelium by Antagonizing FGF Signaling, Developmental Cell, vol.8, issue.4, pp.553-564, 2005.
DOI : 10.1016/j.devcel.2005.02.009

K. Peters, D. Ornitz, S. Werner, and L. Williams, Unique Expression Pattern of the FGF Receptor 3 Gene during Mouse Organogenesis, Developmental Biology, vol.155, issue.2, pp.423-430, 1993.
DOI : 10.1006/dbio.1993.1040

R. F. Gorlin, J. J. Pinborg, and M. M. Cohen, Syndromes of the Head and Neck., Plastic and Reconstructive Surgery, vol.89, issue.1, pp.171-174, 1976.
DOI : 10.1097/00006534-199289010-00039

W. O. Collins and S. S. Choi, Otolaryngologic Manifestations of Achondroplasia, Archives of Otolaryngology???Head & Neck Surgery, vol.133, issue.3
DOI : 10.1001/archotol.133.3.237