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Double Hyperautofluorescent Rings in Patients with USH2A-Retinopathy

Ana Fakin , Maja Šuštar , Jelka Brecelj , Crystel Bonnet , Christine Petit , et al.
Genes, 2019, 10 (12), pp.956. ⟨10.3390/genes10120956⟩
Journal articles hal-02408154v1
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Usher syndrome type 1–associated cadherins shape the photoreceptor outer segment

Cataldo Schietroma , Karine Parain , Amrit Estivalet , Asadollah Aghaie , Jacques Boutet de Monvel , et al.
Journal of Cell Biology, 2017, 216 (6), pp.1849 - 1864. ⟨10.1083/jcb.201612030⟩
Journal articles pasteur-01534475v1
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CIB2, defective in isolated deafness, is key for auditory hair cell mechanotransduction and survival

Vincent Michel , Kevin T Booth , Pranav Patni , Matteo Cortese , Hela Azaiez , et al.
EMBO Molecular Medicine, 2017, 9 (12), pp.1711 - 1731. ⟨10.15252/emmm.201708087⟩
Journal articles hal-01661175v1
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Exploration du rôle des différents domaines C2 de l'otoferline et des isoformes des canaux calciques CaV1.3 dans la transmission synaptique des cellules ciliées auditives

Margot Tertrais
Neurosciences. Université de Bordeaux, 2018. Français. ⟨NNT : 2018BORD0435⟩
Theses tel-02111896v1
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Otogelin, otogelin-like, and stereocilin form links connecting outer hair cell stereocilia to each other and the tectorial membrane

Paul Avan , Sébastien Le Gal , Vincent Michel , Typhaine Dupont , Jean-Pierre Hardelin , et al.
Proceedings of the National Academy of Sciences of the United States of America, 2019, 116 (51), pp.25948-25957. ⟨10.1073/pnas.1902781116⟩
Journal articles pasteur-02860028v1
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Octopus Cells in the Posteroventral Cochlear Nucleus Provide the Main Excitatory Input to the Superior Paraolivary Nucleus

Richard A. Felix Il , Boris Gourévitch , Marcelo Gomez-Alvarez , Sara C. M. Leijon , Enrique Saldana , et al.
Frontiers in Neural Circuits, 2017, 11, pp.37. ⟨10.3389/fncir.2017.00037⟩
Journal articles hal-01556581v1
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Progrès de la thérapie génique : espoirs pour le syndrome d'Usher

Charlotte Calvet , Ghizlene Lahlou , Saaid Safieddine
Médecine/Sciences, 2018, 34 (10), pp.842 - 848. ⟨10.1051/medsci/2018210⟩
Journal articles hal-02365420v1
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Otoferlin acts as a Ca2+ sensor for vesicle fusion and vesicle pool replenishment at auditory hair cell ribbon synapses

Nicolas Michalski , Juan D Goutman , Sarah Marie Auclair , Jacques Boutet de Monvel , Margot Tertrais , et al.
eLife, 2017, 6, pp.e31013. ⟨10.7554/eLife.31013⟩
Journal articles hal-01665122v1
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Clarin‐2 is essential for hearing by maintaining stereocilia integrity and function

Lucy A Dunbar , Pranav Patni , Carlos Aguilar , Philomena Mburu , Laura Corns , et al.
EMBO Molecular Medicine, 2019, 11 (9), pp.e10288. ⟨10.15252/emmm.201910288⟩
Journal articles pasteur-03261798v1
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Triangles bridge the scales: Quantifying cellular contributions to tissue deformation.

Matthias Merkel , Raphael Etournay , Marko Popović , Guillaume Salbreux , Suzanne Eaton , et al.
Physical Review E , 2017, 95 (3), pp.032401. ⟨10.1103/PhysRevE.95.032401⟩
Journal articles pasteur-01545825v1
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Mechanotransduction is required for establishing and maintaining mature inner hair cells and regulating efferent innervation

Laura F Corns , Stuart L Johnson , Terri Roberts , Kishani M Ranatunga , Aenea Hendry , et al.
Nature Communications, 2018, 9 (1), pp.4015. ⟨10.1038/s41467-018-06307-w⟩
Journal articles hal-04159530v1
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Different Ca V 1.3 Channel Isoforms Control Distinct Components of the Synaptic Vesicle Cycle in Auditory Inner Hair Cells

Philippe F.Y. Vincent , Yohan Bouleau , Gilles Charpentier , Alice Emptoz , Saaid Safieddine , et al.
Journal of Neuroscience, 2017, 37 (11), pp.2960-2975. ⟨10.1523/JNEUROSCI.2374-16.2017⟩
Journal articles hal-04159590v1
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Viral transfer of mini-otoferlins partially restores the fast component of exocytosis and uncovers ultrafast endocytosis in auditory hair cells of otoferlin knock-out mice

Margot Tertrais , Yohan Bouleau , Alice Emptoz , Séverin Belleudy , R Bryan Sutton , et al.
Journal of Neuroscience, 2019, 39 (18), pp.3394-3411. ⟨10.1523/JNEUROSCI.1550-18.2018⟩
Journal articles hal-04159459v1
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Local gene therapy durably restores vestibular function in a mouse model of Usher syndrome type 1G

Alice Emptoz , Vincent Michel , Andrea Lelli , Omar Akil , Jacques Boutet de Monvel , et al.
Proceedings of the National Academy of Sciences of the United States of America, 2017, 114 (36), pp.9695 - 9700. ⟨10.1073/pnas.1708894114⟩
Journal articles hal-01661148v1
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A novel PEX1 mutation in a Moroccan family with Zellweger spectrum disorders

Amale Bousfiha , Amina Bakhchane , Hicham Charoute , Zied Riahi , Khalid Snoussi , et al.
Human Genome Variation, 2017, 4 (1), pp.17009. ⟨10.1038/hgv.2017.9⟩
Journal articles pasteur-03219646v1
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Etude du rôle de protéines apparentées aux cadhérines dans le développement des interneurones du cortex auditif

Baptiste Libé-Philippot
Biologie du développement. Université Pierre et Marie Curie - Paris VI, 2017. Français. ⟨NNT : 2017PA066112⟩
Theses tel-01666045v1
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Cochlear outer hair cell horizontal top connectors mediate mature stereocilia bundle mechanics

Alexander X Cartagena-Rivera , Sébastien Le Gal , Kerianne Richards , Elisabeth Verpy , Richard S Chadwick
Science Advances , 2019, 5 (2), pp.eaat9934. ⟨10.1126/sciadv.aat9934⟩
Journal articles hal-02052207v1
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Genes Involved in the Development and Physiology of Both the Peripheral and Central Auditory Systems

Nicolas Michalski , Christine Petit
Annual Review of Neuroscience, 2019, 42, pp.67-86. ⟨10.1146/annurev-neuro-070918-050428⟩
Journal articles pasteur-02874563v1
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Dual AAV-mediated gene therapy restores hearing in a DFNB9 mouse model

Omar Akil , Frank M. Dyka , Charlotte Calvet , Alice Emptoz , Ghizlene Lahlou , et al.
Proceedings of the National Academy of Sciences of the United States of America, 2019, 116 (10), pp.4496-4501. ⟨10.1073/pnas.1817537116⟩
Journal articles hal-02318673v1
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DNABarcodeCompatibility: an R-package for optimizing DNA-barcode combinations in multiplex sequencing experiments

Céline Trébeau , Jacques Boutet de Monvel , Fabienne Wong Jun Tai , Christine Petit , Raphael Etournay
Journal articles pasteur-02175109v1
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Clarin-1 gene transfer rescues auditory synaptopathy in model of Usher syndrome

Didier Dulon , Samantha Papal , Pranav Patni , Matteo Cortese , Philippe F.y Vincent , et al.
Journal of Clinical Investigation, 2018, 128 (8), pp.3382 - 3401. ⟨10.1172/JCI94351⟩
Journal articles pasteur-01858480v1
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Cell dynamics underlying oriented growth of the Drosophila wing imaginal disc

Natalie A Dye , Marko Popović , Stephanie Spannl , Raphael Etournay , Dagmar Kainmüller , et al.
Development (Cambridge, England), 2017, 144 (23), pp.4406 - 4421. ⟨10.1242/dev.155069⟩
Journal articles pasteur-01678565v1
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Vers une thérapie génique de certaines surdités congénitales ?

Jean-Pierre Hardelin , Saaid Safieddine
Médecine/Sciences, 2019, 35 (12), pp.1213-1215. ⟨10.1051/medsci/2019233⟩
Journal articles hal-04159440v1
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Cone degeneration is triggered by the absence of USH1 proteins but prevented by antioxidant treatments

Alix Trouillet , Elisabeth Dubus , Julie Degardin , Amrit Estivalet , Ivana Ivkovic , et al.
Scientific Reports, 2018, 8 (1), pp.1968. ⟨10.1038/s41598-018-20171-0⟩
Journal articles hal-01708801v1
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Stiffness and tension gradients of the hair cell's tip-link complex in the mammalian cochlea

Mélanie Tobin , Atitheb Chaiyasitdhi , Vincent Michel , Nicolas Michalski , Pascal Martin
eLife, 2019, 8, pp.e43473. ⟨10.7554/eLife.43473⟩
Journal articles pasteur-02874559v1
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Clinical and Haplotypic Variability of Slovenian USH2A Patients Homozygous for the c. 11864G>A Nonsense Mutation

Andrej Zupan , Ana Fakin , Saba Battelino , Martina Jarc-Vidmar , Marko Hawlina , et al.
Genes, 2019, 10 (12), pp.1015. ⟨10.3390/genes10121015⟩
Journal articles hal-02417524v1
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Benefits of a contralateral routing of signal device for unilateral Naída CI cochlear implant recipients

Isabelle Mosnier , Ghizlene Lahlou , Jonathan Flament , Nathalie Mathias , Evelyne Ferrary , et al.
European Archives of Oto-Rhino-Laryngology, 2019, 276 (8), pp.2205-2213. ⟨10.1007/s00405-019-05467-9⟩
Journal articles hal-02385205v1