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Items: 8

1.

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.

J Neurosci. 2019 May 1;39(18):3394-3411. doi: 10.1523/JNEUROSCI.1550-18.2018. Epub 2019 Mar 4.

PMID:
30833506
2.

Dual AAV-mediated gene therapy restores hearing in a DFNB9 mouse model.

Akil O, Dyka F, Calvet C, Emptoz A, Lahlou G, Nouaille S, Boutet de Monvel J, Hardelin JP, Hauswirth WW, Avan P, Petit C, Safieddine S, Lustig LR.

Proc Natl Acad Sci U S A. 2019 Feb 19. pii: 201817537. doi: 10.1073/pnas.1817537116. [Epub ahead of print]

3.

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.

J Clin Invest. 2018 Aug 1;128(8):3382-3401. doi: 10.1172/JCI94351. Epub 2018 Jul 9.

4.

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.

Elife. 2017 Nov 7;6. pii: e31013. doi: 10.7554/eLife.31013.

5.

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.

EMBO Mol Med. 2017 Dec;9(12):1711-1731. doi: 10.15252/emmm.201708087.

6.

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.

Proc Natl Acad Sci U S A. 2017 Sep 5;114(36):9695-9700. doi: 10.1073/pnas.1708894114. Epub 2017 Aug 23.

7.

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.

J Neurosci. 2017 Mar 15;37(11):2960-2975. doi: 10.1523/JNEUROSCI.2374-16.2017. Epub 2017 Feb 13.

8.

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.

Cell. 2015 Nov 5;163(4):894-906. doi: 10.1016/j.cell.2015.10.023.

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