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2024In utero adeno-associated virus (AAV)-mediated gene delivery targeting sensory and supporting cells in the embryonic mouse inner ear., PLoS One 2024 ; 19(7): e0305742.
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2024Extended time frame for restoring inner ear function through gene therapy in Usher1G preclinical model, JCI Insight, 2024, 9 (3), pp.e169504. ⟨10.1172/jci.insight.169504⟩.
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2023Recent advances and future challenges in gene therapy for hearing loss., R Soc Open Sci 2023 Jun; 10(6): 230644.
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2023Deafness: from genetic architecture to gene therapy, Nature Reviews Genetics, 2023, ⟨10.1038/s41576-023-00597-7⟩.
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2023Towards the Clinical Application of Gene Therapy for Genetic Inner Ear Diseases., J Clin Med 2023 Jan; 12(3): .
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2022The SNARE protein SNAP-25 is required for normal exocytosis at auditory hair cell ribbon synapses., iScience 2022 Dec; 25(12): 105628.
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2019Viral Transfer of Mini-Otoferlins Partially Restores the Fast Component of Exocytosis and Uncovers Ultrafast Endocytosis in Auditory Hair Cells of Otoferlin Knock-Out Mice., J Neurosci 2019 May; 39(18): 3394-3411.
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2019Dual AAV-mediated gene therapy restores hearing in a DFNB9 mouse model., Proc Natl Acad Sci U S A 2019 Mar; 116(10): 4496-4501.
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2018Mechanotransduction is required for establishing and maintaining mature inner hair cells and regulating efferent innervation., Nat Commun 2018 Oct; 9(1): 4015.
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2018Clarin-1 gene transfer rescues auditory synaptopathy in model of Usher syndrome., J Clin Invest 2018 Aug; 128(8): 3382-3401.
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2017Otoferlin acts as a Ca2+ sensor for vesicle fusion and vesicle pool replenishment at auditory hair cell ribbon synapses., Elife 2017 Nov; 6(): .
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2017Local gene therapy durably restores vestibular function in a mouse model of Usher syndrome type 1G., Proc Natl Acad Sci U S A 2017 Sep; 114(36): 9695-9700.
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2017Different Ca V 1.3 Channel Isoforms Control Distinct Components of the Synaptic Vesicle Cycle in Auditory Inner Hair Cells, Journal of Neuroscience, 2017, 37 (11), pp.2960-2975. ⟨10.1523/JNEUROSCI.2374-16.2017⟩.
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2015Hypervulnerability to Sound Exposure through Impaired Adaptive Proliferation of Peroxisomes., Cell 2015 Nov; 163(4): 894-906.
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2014Exocytotic Machineries of Vestibular Type I and Cochlear Ribbon Synapses Display Similar Intrinsic Otoferlin-Dependent Ca2+ Sensitivity But a Different Coupling to Ca2+ Channels, Journal of Neuroscience, 2014, 34 (33), pp.10853-10869. ⟨10.1523/JNEUROSCI.0947-14.2014⟩.
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2012The auditory hair cell ribbon synapse: from assembly to function., Annu Rev Neurosci 2012 ; 35(): 509-28.
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2010Control of exocytosis by synaptotagmins and otoferlin in auditory hair cells., J Neurosci 2010 Oct; 30(40): 13281-90.
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2009Myosin VI is required for the proper maturation and function of inner hair cell ribbon synapses., Hum Mol Genet 2009 Dec; 18(23): 4615-28.
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2009Otoferlin is critical for a highly sensitive and linear calcium-dependent exocytosis at vestibular hair cell ribbon synapses., J Neurosci 2009 Aug; 29(34): 10474-87.
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2008αII-βV spectrin bridges the plasma membrane and cortical lattice in the lateral wall of the auditory outer hair cells, Journal of Cell Science, 2008, 121 (20), pp.3347-3356. ⟨10.1242/jcs.028134⟩.
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2008Calcium- and otoferlin-dependent exocytosis by immature outer hair cells., J Neurosci 2008 Feb; 28(8): 1798-803.
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2006Otoferlin, defective in a human deafness form, is essential for exocytosis at the auditory ribbon synapse., Cell 2006 Oct; 127(2): 277-89.
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2000KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway., Proc Natl Acad Sci U S A 2000 Apr; 97(8): 4333-8.
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