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Dual AAV-mediated gene therapy restores hearing in a DFNB9 mouse model.
Proc Natl Acad Sci U S A. 2019 Mar 5;116(10):4496-4501. doi: 10.1073/pnas.1817537116. Epub 2019 Feb 19.
Proc Natl Acad Sci U S A. 2019.
PMID: 30782832
Free PMC article.
Local gene therapy durably restores vestibular function in a mouse model of Usher syndrome type 1G.
Emptoz A, Michel V, Lelli A, Akil O, Boutet de Monvel J, Lahlou G, Meyer A, Dupont T, Nouaille S, Ey E, Franca de Barros F, Beraneck M, Dulon D, Hardelin JP, Lustig L, Avan P, Petit C, Safieddine S.
Emptoz A, et al.
Proc Natl Acad Sci U S A. 2017 Sep 5;114(36):9695-9700. doi: 10.1073/pnas.1708894114. Epub 2017 Aug 23.
Proc Natl Acad Sci U S A. 2017.
PMID: 28835534
Free PMC article.
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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.
Tertrais M, Bouleau Y, Emptoz A, Belleudy S, Sutton RB, Petit C, Safieddine S, Dulon D.
Tertrais M, et al. Among authors: emptoz a.
J Neurosci. 2019 May 1;39(18):3394-3411. doi: 10.1523/JNEUROSCI.1550-18.2018. Epub 2019 Mar 4.
J Neurosci. 2019.
PMID: 30833506
Free PMC article.
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Clarin-1 gene transfer rescues auditory synaptopathy in model of Usher syndrome.
Dulon D, Papal S, Patni P, Cortese M, Vincent PF, Tertrais M, Emptoz A, Tlili A, Bouleau Y, Michel V, Delmaghani S, Aghaie A, Pepermans E, Alegria-Prevot O, Akil O, Lustig L, Avan P, Safieddine S, Petit C, El-Amraoui A.
Dulon D, et al. Among authors: emptoz a.
J Clin Invest. 2018 Aug 1;128(8):3382-3401. doi: 10.1172/JCI94351. Epub 2018 Jul 9.
J Clin Invest. 2018.
PMID: 29985171
Free PMC article.
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CIB2, defective in isolated deafness, is key for auditory hair cell mechanotransduction and survival.
Michel V, Booth KT, Patni P, Cortese M, Azaiez H, Bahloul A, Kahrizi K, Labbé M, Emptoz A, Lelli A, Dégardin J, Dupont T, Aghaie A, Oficjalska-Pham D, Picaud S, Najmabadi H, Smith RJ, Bowl MR, Brown SD, Avan P, Petit C, El-Amraoui A.
Michel V, et al. Among authors: emptoz a.
EMBO Mol Med. 2017 Dec;9(12):1711-1731. doi: 10.15252/emmm.201708087.
EMBO Mol Med. 2017.
PMID: 29084757
Free PMC article.
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Otoferlin acts as a Ca2+ sensor for vesicle fusion and vesicle pool replenishment at auditory hair cell ribbon synapses.
Michalski N, Goutman JD, Auclair SM, Boutet de Monvel J, Tertrais M, Emptoz A, Parrin A, Nouaille S, Guillon M, Sachse M, Ciric D, Bahloul A, Hardelin JP, Sutton RB, Avan P, Krishnakumar SS, Rothman JE, Dulon D, Safieddine S, Petit C.
Michalski N, et al. Among authors: emptoz a.
Elife. 2017 Nov 7;6:e31013. doi: 10.7554/eLife.31013.
Elife. 2017.
PMID: 29111973
Free PMC article.
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Hypervulnerability to Sound Exposure through Impaired Adaptive Proliferation of Peroxisomes.
Delmaghani S, Defourny J, Aghaie A, Beurg M, Dulon D, Thelen N, Perfettini I, Zelles T, Aller M, Meyer A, Emptoz A, Giraudet F, Leibovici M, Dartevelle S, Soubigou G, Thiry M, Vizi ES, Safieddine S, Hardelin JP, Avan P, Petit C.
Delmaghani S, et al. Among authors: emptoz a.
Cell. 2015 Nov 5;163(4):894-906. doi: 10.1016/j.cell.2015.10.023.
Cell. 2015.
PMID: 26544938
Free article.
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Different CaV1.3 Channel Isoforms Control Distinct Components of the Synaptic Vesicle Cycle in Auditory Inner Hair Cells.
Vincent PF, Bouleau Y, Charpentier G, Emptoz A, Safieddine S, Petit C, Dulon D.
Vincent PF, et al. Among authors: emptoz a.
J Neurosci. 2017 Mar 15;37(11):2960-2975. doi: 10.1523/JNEUROSCI.2374-16.2017. Epub 2017 Feb 13.
J Neurosci. 2017.
PMID: 28193694
Free PMC article.
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