C. Bonnet and A. El-amraoui, Usher syndrome (sensorineural deafness and retinitis pigmentosa), Current Opinion in Neurology, vol.25, issue.1, pp.42-49, 2012.
DOI : 10.1097/WCO.0b013e32834ef8b2

C. Bowes, T. Li, M. Danciger, L. C. Baxter, M. L. Applebury et al., Retinal degeneration in the rd mouse is caused by a defect in the ?? subunit of rod cGMP-phosphodiesterase, Nature, vol.347, issue.6294, pp.677-680, 1990.
DOI : 10.1038/347677a0

P. K. Brown, I. R. Gibbons, and G. Wald, THE VISUAL CELLS AND VISUAL PIGMENT OF THE MUDPUPPY, NECTURUS, The Journal of Cell Biology, vol.19, issue.1, pp.79-106, 1963.
DOI : 10.1083/jcb.19.1.79

E. Caberlotto, V. Michel, I. Foucher, A. Bahloul, R. J. Goodyear et al., Usher type 1G protein sans is a critical component of the tip-link complex, a structure controlling actin polymerization in stereocilia, Proc. Natl. Acad. Sci. USA, pp.5825-5830, 2011.
DOI : 10.1073/pnas.1017114108
URL : https://hal.archives-ouvertes.fr/pasteur-01472844

A. J. Caride, A. R. Penheiter, A. G. Filoteo, Z. Bajzer, A. Enyedi et al., The Plasma Membrane Calcium Pump Displays Memory of Past Calcium Spikes: DIFFERENCES BETWEEN ISOFORMS 2b AND 4b, Journal of Biological Chemistry, vol.276, issue.43, pp.39797-39804, 2001.
DOI : 10.1074/jbc.M104380200

A. I. Cohen, H. P. Scholl, P. Issa, E. Becirovic, J. Lamprecht et al., VERTEBRATE RETINAL CELLS AND THEIR ORGANIZATION, Biological Reviews, vol.16, issue.4, pp.427-459203, 1963.
DOI : 10.1083/jcb.17.2.279

M. S. Eckmiller, Cone outer segment morphogenesis: taper change and distal invaginations, The Journal of Cell Biology, vol.105, issue.5, pp.2267-2277, 1987.
DOI : 10.1083/jcb.105.5.2267

A. El-amraoui and C. Petit, Usher I syndrome: unravelling the mechanisms that underlie the cohesion of the growing hair bundle in inner ear sensory cells, Journal of Cell Science, vol.118, issue.20, pp.4593-4603, 2005.
DOI : 10.1242/jcs.02636

A. El-amraoui and C. Petit, Cadherins as Targets for Genetic Diseases, Cold Spring Harbor Perspectives in Biology, vol.2, issue.1, p.3095, 2010.
DOI : 10.1101/cshperspect.a003095

A. El-amraoui, I. Sahly, S. Picaud, J. Sahel, M. Abitbol et al., Human Usher 1B/mouse shaker-1: the retinal phenotype discrepancy explained by the presence/absence of myosin VIIA in the photoreceptor cells, Human Molecular Genetics, vol.5, issue.8, pp.1171-1178, 1996.
DOI : 10.1093/hmg/5.8.1171

A. El-amraoui, J. Schonn, P. Küssel-andermann, S. Blanchard, C. Desnos et al., MyRIP, a novel Rab effector, enables myosin VIIa recruitment to retinal melanosomes, EMBO reports, vol.3, issue.5, pp.463-470, 2002.
DOI : 10.1093/embo-reports/kvf090

H. M. Elledge, P. Kazmierczak, P. Clark, J. S. Joseph, A. Kolatkar et al., Structure of the N terminus of cadherin 23 reveals a new adhesion mechanism for a subset of cadherin superfamily members, Proc. Natl. Acad. Sci. USA, pp.10708-10712, 2010.
DOI : 10.1073/pnas.1006284107

J. D. Eudy, M. D. Weston, S. Yao, D. M. Hoover, H. L. Rehm et al., Mutation of a Gene Encoding a Protein with Extracellular Matrix Motifs in Usher Syndrome Type IIa, Science, vol.280, issue.5370, pp.1753-1757, 1998.
DOI : 10.1126/science.280.5370.1753

R. Flores-guevara, F. Renault, N. Loundon, S. Marlin, B. Pelosse et al., Usher syndrome type 1: Early detection of electroretinographic changes, European Journal of Paediatric Neurology, vol.13, issue.6, pp.505-507, 2009.
DOI : 10.1016/j.ejpn.2008.10.002

D. N. Furness, Y. Katori, B. Kumar, and C. M. Hackney, The dimensions and structural attachments of tip links in mammalian cochlear hair cells and the effects of exposure to different levels of extracellular calcium, Neuroscience, vol.154, issue.1, pp.10-21, 2008.
DOI : 10.1016/j.neuroscience.2008.02.010

M. Giacomello, A. De-mario, R. Lopreiato, S. Primerano, M. Campeol et al., Mutations in PMCA2 and hereditary deafness: A molecular analysis of the pump defect, Cell Calcium, vol.50, issue.6, pp.569-576, 2011.
DOI : 10.1016/j.ceca.2011.09.004

D. Gibbs, S. M. Azarian, C. Lillo, J. Kitamoto, A. E. Klomp et al., Role of myosin VIIa and Rab27a in the motility and localization of RPE melanosomes, Journal of Cell Science, vol.117, issue.26, pp.6473-6483, 2004.
DOI : 10.1242/jcs.01580

R. J. Goodyear and G. P. Richardson, A novel antigen sensitive to calcium chelation that is associated with the tip links and kinocilial links of sensory hair bundles, J. Neurosci, vol.23, pp.4878-4887, 2003.

M. Grati and B. Kachar, Myosin VIIa and sans localization at stereocilia upper tip-link density implicates these Usher syndrome proteins in Disruption of calyceal process?outer segment links by treatment with subtilisin and BAPTA Adult Xenopus eyes were, 2011.

A. Adato, V. Michel, Y. Kikkawa, J. Reiners, K. N. Alagramam et al., Interactions in the network of Usher syndrome type 1 proteins, Human Molecular Genetics, vol.14, issue.3, pp.347-356, 2005.
DOI : 10.1093/hmg/ddi031

Z. M. Ahmed, S. Riazuddin, J. Ahmad, S. L. Bernstein, Y. Guo et al., PCDH15 is expressed in the neurosensory epithelium of the eye and ear and mutant alleles are responsible for both USH1F and DFNB23, Human Molecular Genetics, vol.12, issue.24, pp.3215-3223, 2003.
DOI : 10.1093/hmg/ddg358

Z. M. Ahmed, S. Kjellstrom, R. J. Haywood-watson, R. A. Bush, L. L. Hampton et al., Double homozygous waltzer and Ames waltzer mice provide no evidence of retinal degeneration, Mol. Vis, vol.14, pp.2227-2236, 2008.

A. Bahloul, V. Michel, J. P. Hardelin, S. Nouaille, S. Hoos et al., Cadherin-23, myosin VIIa and harmonin, encoded by Usher syndrome type I genes, form a ternary complex and interact with membrane phospholipids, Human Molecular Genetics, vol.19, issue.18, pp.3557-3565, 2010.
DOI : 10.1093/hmg/ddq271

S. L. Ball, D. Bardenstein, and K. N. Alagramam, Assessment of Retinal Structure and Function in Ames Waltzer Mice, Investigative Opthalmology & Visual Science, vol.44, issue.9, 2003.
DOI : 10.1167/iovs.02-1009

B. Boëda, A. El-amraoui, A. Bahloul, R. Goodyear, L. Daviet et al., Myosin VIIa, harmonin and cadherin 23, three Usher I gene products that cooperate to shape the sensory hair cell bundle, The EMBO Journal, vol.21, issue.24, pp.6689-6699, 2002.
DOI : 10.1093/emboj/cdf689

J. Lin-jones and B. Burnside, Retina-Specific Protein Fascin 2 Is an Actin Cross-linker Associated with Actin Bundles in Photoreceptor Inner Segments and Calycal Processes, Investigative Opthalmology & Visual Science, vol.48, issue.3, pp.1380-138806, 2007.
DOI : 10.1167/iovs.06-0763

J. Lin-jones, E. Parker, M. Wu, A. Dosé, and B. Burnside, Myosin 3A transgene expression produces abnormal actin filament bundles in transgenic Xenopus laevis rod photoreceptors, Journal of Cell Science, vol.117, issue.24, pp.5825-5834, 2004.
DOI : 10.1242/jcs.01512

X. Liu, G. Vansant, I. P. Udovichenko, U. Wolfrum, and D. S. Williams, Myosin VIIa, the product of the Usher 1B syndrome gene, is concentrated in the connecting cilia of photoreceptor cells, 3<240::AID-CM6>3.0.CO, pp.240-2521097, 1997.

X. Liu, B. Ondek, and D. S. Williams, Mutant myosin VIIa causes defective melanosome distribution in the RPE of shaker-1 mice, Nature Genetics, vol.19, issue.2, pp.117-118470, 1998.
DOI : 10.1038/470

X. Liu, I. P. Udovichenko, S. D. Brown, K. P. Steel, and D. S. Williams, Myosin VIIa participates in opsin transport through the photoreceptor cilium, J. Neurosci, vol.19, pp.6267-6274, 1999.

T. Maerker, E. Van-wijk, N. Overlack, F. F. Kersten, J. Mcgee et al., A novel Usher protein network at the periciliary reloading point between molecular transport machineries in vertebrate photoreceptor cells, Human Molecular Genetics, vol.17, issue.1, pp.71-86, 2008.
DOI : 10.1093/hmg/ddm285

E. Malm, V. Ponjavic, C. Möller, W. J. Kimberling, and S. Andréasson, Phenotypes in Defined Genotypes Including Siblings with Usher Syndrome, Ophthalmic Genetics, vol.37, issue.10, pp.65-74, 2011.
DOI : 10.1167/iovs.03-0514

E. Malm, V. Ponjavic, C. Möller, W. J. Kimberling, E. S. Stone et al., Alteration of rod and cone function in children with Usher syndrome, European Journal of Ophthalmology, vol.21, issue.1, pp.30-38, 2011.
DOI : 10.5301/EJO.2010.5433

T. M. Mayhew, C. Mühlfeld, D. Vanhecke, and M. Ochs, A review of recent methods for efficiently quantifying immunogold and other nanoparticles using TEM sections through cells, tissues and organs, Annals of Anatomy - Anatomischer Anzeiger, vol.191, issue.2, 2009.
DOI : 10.1016/j.aanat.2008.11.001

P. Mburu, M. Mustapha, A. Varela, D. Weil, A. El-amraoui et al., Defects in whirlin, a PDZ domain molecule involved in stereocilia elongation, cause deafness in the whirler mouse and families with DFNB31, Nature Genetics, vol.34, issue.4, pp.421-428, 2003.
DOI : 10.1038/ng1208

N. Michalski, V. Michel, E. Caberlotto, G. M. Lefèvre, A. F. Van-aken et al., Harmonin-b, an actin-binding scaffold protein, is involved in the adaptation of mechanoelectrical transduction by sensory hair cells, Pfl??gers Archiv - European Journal of Physiology, vol.82, issue.1, pp.115-130, 2009.
DOI : 10.1007/s00424-009-0711-x

V. Michel, R. J. Goodyear, D. Weil, W. Marcotti, I. Perfettini et al., Cadherin 23 is a component of the transient lateral links in the developing hair bundles of cochlear sensory cells, Developmental Biology, vol.280, issue.2, pp.281-294, 2005.
DOI : 10.1016/j.ydbio.2005.01.014

P. D. Nieuwkoop and J. Faber, Normal table of Xenopus laevis, 1967.

O. Connor, P. , and B. Burnside, Actin-dependent cell elongation in teleost retinal rods: requirement for actin filament assembly, The Journal of Cell Biology, vol.89, issue.3, pp.517-524, 1981.
DOI : 10.1083/jcb.89.3.517

K. Pagh-roehl, E. Wang, and B. Burnside, Shortening of the calycal process actin cytoskeleton is correlated with myoid elongation in teleost rods, Experimental Eye Research, vol.55, issue.5, pp.735-7460014, 1992.
DOI : 10.1016/0014-4835(92)90178-U

L. Pan and M. Zhang, Structures of Usher Syndrome 1 Proteins and Their Complexes, Physiology, vol.27, issue.1, pp.25-42, 2011.
DOI : 10.1152/physiol.00037.2011

L. Pan, J. Yan, L. Wu, and M. Zhang, Assembling stable hair cell tip link complex via multidentate interactions between harmonin and cadherin 23, Proc. Natl. Acad. Sci. USA, pp.5575-5580, 2009.
DOI : 10.1073/pnas.0901819106

C. Petit, : From Genetics to Pathogenesis, Annual Review of Genomics and Human Genetics, vol.2, issue.1, pp.271-297, 2001.
DOI : 10.1146/annurev.genom.2.1.271

C. Petit and G. P. Richardson, Linking genes underlying deafness to hair-bundle development and function, Nature Neuroscience, vol.249, issue.6, pp.703-710, 2009.
DOI : 10.1038/nn.2330

J. Reiners and U. Wolfrum, Molecular Analysis of the Supramolecular Usher Protein Complex in the Retina, Proc. Natl. Acad. Sci. USA, pp.349-35311476, 2006.
DOI : 10.1007/0-387-32442-9_49

N. Grillet, W. Xiong, A. Reynolds, P. Kazmierczak, T. Sato et al., Harmonin Mutations Cause Mechanotransduction Defects in Cochlear Hair Cells, Neuron, vol.62, issue.3, pp.375-387, 2009.
DOI : 10.1016/j.neuron.2009.04.006

T. Hasson, M. B. Heintzelman, J. Santos-sacchi, D. P. Corey, and M. S. Mooseker, Expression in cochlea and retina of myosin VIIa, the gene product defective in Usher syndrome type 1B., Proc. Natl. Acad. Sci. USA, pp.9815-9819, 1995.
DOI : 10.1073/pnas.92.21.9815

R. J. Haywood-watson, Z. M. Ii, S. Ahmed, R. A. Kjellstrom, Y. Bush et al., Ames Waltzer deaf mice have reduced electroretinogram amplitudes and complex alternative splicing of Pcdh15 transcripts, Invest. Ophthalmol. Vis, 2006.

S. M. Heissler and D. J. Manstein, Functional characterization of the human myosin-7a motor domain, Cellular and Molecular Life Sciences, vol.280, issue.2, pp.299-311, 2012.
DOI : 10.1007/s00018-011-0749-8

A. Henn, E. M. De, and L. Cruz, Vertebrate Myosin VIIb Is a High Duty Ratio Motor Adapted for Generating and Maintaining Tension, Journal of Biological Chemistry, vol.280, issue.47, pp.39665-39676, 2005.
DOI : 10.1074/jbc.M507667200

A. A. Hirano, J. H. Brandstätter, C. W. Morgans, and N. C. Brecha, SNAP25 expression in mammalian retinal horizontal cells, The Journal of Comparative Neurology, vol.34, issue.5, pp.972-988, 2011.
DOI : 10.1002/cne.22562

D. Höfer and D. Drenckhahn, Molecular heterogeneity of the actin filament cytoskeleton associated with microvilli of photoreceptors, M???ller's glial cells and pigment epithelial cells of the retina, Histochemistry, vol.33, issue.12, pp.29-35, 1993.
DOI : 10.1007/BF00268017

A. Inoue and M. Ikebe, Characterization of the Motor Activity of Mammalian Myosin VIIA, Journal of Biological Chemistry, vol.278, issue.7, pp.5478-5487, 2003.
DOI : 10.1074/jbc.M210489200

S. G. Jacobson, A. V. Cideciyan, D. Gibbs, A. Sumaroka, A. J. Roman et al., Mutations, Investigative Opthalmology & Visual Science, vol.52, issue.11, pp.7924-793611, 2011.
DOI : 10.1167/iovs.11-8313
URL : https://hal.archives-ouvertes.fr/hal-00552398

K. R. Johnson, L. H. Gagnon, L. S. Webb, L. L. Peters, N. L. Hawes et al., Mouse models of USH1C and DFNB18: phenotypic and molecular analyses of two new spontaneous mutations of the Ush1c gene, Human Molecular Genetics, vol.12, issue.23, pp.3075-3086, 2003.
DOI : 10.1093/hmg/ddg332

P. Kazmierczak, H. Sakaguchi, J. Tokita, E. M. Wilson-kubalek, R. A. Milligan et al., Cadherin 23 and protocadherin 15 interact to form tip-link filaments in sensory hair cells, Nature, vol.15, issue.7158, pp.87-91, 2007.
DOI : 10.1038/nature06091

B. Kennedy and J. Malicki, What drives cell morphogenesis: A look inside the vertebrate photoreceptor, Developmental Dynamics, vol.28, issue.Spec No 1, pp.2115-2138, 2009.
DOI : 10.1002/dvdy.22010

A. Lagziel, Z. M. Ahmed, J. M. Schultz, R. J. Morell, I. A. Belyantseva et al., Spatiotemporal pattern and isoforms of cadherin 23 in wild type and waltzer mice during inner ear hair cell development, Developmental Biology, vol.280, issue.2, pp.295-306, 2005.
DOI : 10.1016/j.ydbio.2005.01.015

A. Lagziel, N. Overlack, S. L. Bernstein, R. J. Morell, U. Wolfrum et al., Expression of cadherin 23 isoforms is not conserved: implications for a mouse model of Usher syndrome type 1D, Mol. Vis, vol.15, pp.1843-1857, 2009.

G. Lefèvre, V. Michel, D. Weil, L. Lepelletier, E. Bizard et al., A core cochlear phenotype in USH1 mouse mutants implicates fibrous links of the hair bundle in its cohesion, orientation and differential growth, Development, vol.135, issue.8, pp.1427-1437, 2008.
DOI : 10.1242/dev.012922

K. Legendre, S. Safieddine, P. Küssel-andermann, C. Petit, and A. El-amraoui, ??II-??V spectrin bridges the plasma membrane and cortical lattice in the lateral wall of the auditory outer hair cells, Journal of Cell Science, vol.121, issue.20, pp.3347-3356, 2008.
DOI : 10.1242/jcs.028134

J. J. Lentz, W. C. Gordon, H. E. Farris, G. H. Macdonald, D. E. Cunningham et al., Deafness and retinal degeneration in a novel USH1C knock-in mouse model, Developmental Neurobiology, vol.74, issue.4, pp.253-267, 2010.
DOI : 10.1002/dneu.20771

R. T. Libby and K. P. Steel, Electroretinographic anomalies in mice with mutations in Myo7a, the gene involved in human Usher syndrome type 1B, Invest. Ophthalmol. Vis. Sci, vol.42, pp.770-778, 2001.

R. T. Libby, J. Kitamoto, R. H. Holme, D. S. Williams, and K. P. Steel, Cdh23 mutations in the mouse are associated with retinal dysfunction but not retinal degeneration, Experimental Eye Research, vol.77, issue.6, pp.731-739, 2003.
DOI : 10.1016/j.exer.2003.07.007

J. Reiners, B. Reidel, A. El-amraoui, B. Boëda, I. Huber et al., Differential Distribution of Harmonin Isoforms and Their Possible Role in Usher-1 Protein Complexes in Mammalian Photoreceptor Cells, Investigative Opthalmology & Visual Science, vol.44, issue.11, pp.5006-501503, 2003.
DOI : 10.1167/iovs.03-0483

J. Reiners, T. Märker, K. Jürgens, B. Reidel, and U. Wolfrum, Photoreceptor expression of the Usher syndrome type 1 protein protocadherin 15 (USH1F) and its interaction with the scaffold protein harmonin (USH1C), 2005.

G. P. Richardson, J. B. De-monvel, and C. Petit, How the Genetics of Deafness Illuminates Auditory Physiology, Annual Review of Physiology, vol.73, issue.1, pp.311-334, 2011.
DOI : 10.1146/annurev-physiol-012110-142228

J. Siemens, P. Kazmierczak, A. Reynolds, M. Sticker, A. Littlewood-evans et al., The Usher syndrome proteins cadherin 23 and harmonin form a complex by means of PDZ-domain interactions, Proc. Natl. Acad, 2002.
DOI : 10.1073/pnas.232579599

M. Sotomayor, W. A. Weihofen, R. Gaudet, and D. P. Corey, Structural Determinants of Cadherin-23 Function in Hearing and Deafness, Neuron, vol.66, issue.1, pp.85-100, 2010.
DOI : 10.1016/j.neuron.2010.03.028

R. H. Steinberg, S. K. Fisher, and D. H. Anderson, Disc morphogenesis in vertebrate photoreceptors, The Journal of Comparative Neurology, vol.42, issue.3, pp.501-508, 1980.
DOI : 10.1002/cne.901900307

C. H. Sung and J. Z. Chuang, The cell biology of vision, The Journal of Cell Biology, vol.28, issue.6, pp.953-963, 2010.
DOI : 10.1073/pnas.0637349100

D. Weil, A. El-amraoui, S. Masmoudi, M. Mustapha, Y. Kikkawa et al., Usher syndrome type I G (USH1G) is caused by mutations in the gene encoding SANS, a protein that associates with the USH1C protein, harmonin, Human Molecular Genetics, vol.12, issue.5, pp.463-471357, 2003.
DOI : 10.1093/hmg/ddg051

D. S. Williams, K. A. Linberg, D. K. Vaughan, R. N. Fariss, and S. K. Fisher, Disruption of microfilament organization and deregulation of disk membrane morphogenesis by cytochalasin D in rod and cone photoreceptors, The Journal of Comparative Neurology, vol.15, issue.2, pp.161-176, 1988.
DOI : 10.1002/cne.902720202

D. S. Williams, T. S. Aleman, C. Lillo, V. S. Lopes, L. C. Hughes et al., -Mutant Mice and Human USH1C, Investigative Opthalmology & Visual Science, vol.50, issue.8, pp.3881-388908, 2009.
DOI : 10.1167/iovs.08-3358
URL : https://hal.archives-ouvertes.fr/hal-00409061

L. Wu, L. Pan, Z. Wei, and M. Zhang, Structure of MyTH4-FERM Domains in Myosin VIIa Tail Bound to Cargo, Science, vol.331, issue.6018, pp.757-760, 2011.
DOI : 10.1126/science.1198848

H. Yagi, H. Tokano, M. Maeda, T. Takabayashi, T. Nagano et al., Vlgr1 is required for proper stereocilia maturation of cochlear hair cells, Genes to Cells, vol.33, issue.2, pp.235-250, 2007.
DOI : 10.1016/0006-8993(96)00147-3

J. Yan, L. Pan, X. Chen, L. Wu, and M. Zhang, The structure of the harmonin/sans complex reveals an unexpected interaction mode of the two Usher syndrome proteins, Proc. Natl. Acad. Sci. USA, pp.4040-4045, 2010.
DOI : 10.1073/pnas.0911385107

L. Zheng, G. Sekerková, K. Vranich, L. G. Tilney, E. Mugnaini et al., The Deaf Jerker Mouse Has a Mutation in the Gene Encoding the Espin Actin-Bundling Proteins of Hair Cell Stereocilia and Lacks Espins, Cell, vol.102, issue.3, pp.377-385, 2000.
DOI : 10.1016/S0092-8674(00)00042-8