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Select item 72298501.

Fetal antisense oligonucleotide therapy for congenital deafness and vestibular dysfunction

Lingyan Wang, J Beth Kempton, Han Jiang, Francine M Jodelka, Alev M Brigande, Rachel A Dumont, Frank Rigo, Jennifer J Lentz, Michelle L Hastings, John V Brigande

Nucleic Acids Res. 2020 May 21; 48(9): 5065–5080. Published online 2020 Apr 6. doi: 10.1093/nar/gkaa194

PMCID:: PMC7229850

Article PDF–8.7M

Select item 72270282.

Mouse Models of Inherited Retinal Degeneration with Photoreceptor Cell Loss

Gayle B. Collin, Navdeep Gogna, Bo Chang, Nattaya Damkham, Jai Pinkney, Lillian F. Hyde, Lisa Stone, Jürgen K. Naggert, Patsy M. Nishina, Mark P. Krebs

Cells. 2020 Apr; 9(4): 931. Published online 2020 Apr 10. doi: 10.3390/cells9040931

PMCID:: PMC7227028

Article PDF–5.4M

Select item 69260403.

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, Christine Petit, Elisabeth Verpy

Proc Natl Acad Sci U S A. 2019 Dec 17; 116(51): 25948–25957. Published online 2019 Nov 27. doi: 10.1073/pnas.1902781116

PMCID:: PMC6926040

Article PDF–3.3M

Select item 67286044.

Clarin‐2 is essential for hearing by maintaining stereocilia integrity and function

Lucy A Dunbar, Pranav Patni, Carlos Aguilar, Philomena Mburu, Laura Corns, Helena RR Wells, Sedigheh Delmaghani, Andrew Parker, Stuart Johnson, Debbie Williams, Christopher T Esapa, Michelle M Simon, Lauren Chessum, Sherylanne Newton, Joanne Dorning, Prashanthini Jeyarajan, Susan Morse, Andrea Lelli, Gemma F Codner, Thibault Peineau, Suhasini R Gopal, Kumar N Alagramam, Ronna Hertzano, Didier Dulon, Sara Wells, Frances M Williams, Christine Petit, Sally J Dawson, Steve DM Brown, Walter Marcotti, Aziz El‐Amraoui, Michael R Bowl

EMBO Mol Med. 2019 Sep; 11(9): e10288. Published online 2019 Aug 26. doi: 10.15252/emmm.201910288

PMCID:: PMC6728604

Article PDF–10M

Select item 64595105.

Mouse screen reveals multiple new genes underlying mouse and human hearing loss

Neil J. Ingham, Selina A. Pearson, Valerie E. Vancollie, Victoria Rook, Morag A. Lewis, Jing Chen, Annalisa Buniello, Elisa Martelletti, Lorenzo Preite, Chi Chung Lam, Felix D. Weiss, Zӧe Powis, Pim Suwannarat, Christopher J. Lelliott, Sally J. Dawson, Jacqueline K. White, Karen P. Steel

PLoS Biol. 2019 Apr; 17(4): e3000194. Published online 2019 Apr 11. doi: 10.1371/journal.pbio.3000194

PMCID:: PMC6459510

Article PDF–5.1M

Select item 61673186.

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, Federico Ceriani, Saaid Safieddine, Karen P. Steel, Andy Forge, Christine Petit, David N. Furness, Corné J. Kros, Walter Marcotti

Nat Commun. 2018; 9: 4015. Published online 2018 Oct 1. doi: 10.1038/s41467-018-06307-w

PMCID:: PMC6167318

Article PDF–3.9M

Select item 60756027.

The roles of USH1 proteins and PDZ domain-containing USH proteins in USH2 complex integrity in cochlear hair cells

Junhuang Zou, Qian Chen, Ali Almishaal, Pranav Dinesh Mathur, Tihua Zheng, Cong Tian, Qing Y. Zheng, Jun Yang

Hum Mol Genet. 2017 Feb 1; 26(3): 624–636. Published online 2016 Dec 28. doi: 10.1093/hmg/ddw421

PMCID:: PMC6075602

Article PDF–1.7M

Select item 58862448.

Rescue of peripheral vestibular function in Usher syndrome mice using a splice-switching antisense oligonucleotide

Sarath Vijayakumar, Frederic F. Depreux, Francine M. Jodelka, Jennifer J. Lentz, Frank Rigo, Timothy A. Jones, Michelle L. Hastings

Hum Mol Genet. 2017 Sep 15; 26(18): 3482–3494. Published online 2017 Jun 19. doi: 10.1093/hmg/ddx234

PMCID:: PMC5886244

Article PDF–424K

Select item 57924409.

Cone degeneration is triggered by the absence of USH1 proteins but prevented by antioxidant treatments

Alix Trouillet, Elisabeth Dubus, Julie Dégardin, Amrit Estivalet, Ivana Ivkovic, David Godefroy, Diego García-Ayuso, Manuel Simonutti, Iman Sahly, José A. Sahel, Aziz El-Amraoui, Christine Petit, Serge Picaud

Sci Rep. 2018; 8: 1968. Published online 2018 Jan 31. doi: 10.1038/s41598-018-20171-0

PMCID:: PMC5792440

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Select item 568389810.

Antisense oligonucleotide therapy rescues disruptions in organization of exploratory movements associated with Usher syndrome type 1C in mice

Tia N. Donaldson, Kelsey T. Jennings, Lucia A. Cherep, Adam M. McNeela, Frederic F. Depreux, Francine M. Jodelka, Michelle L. Hastings, Douglas G. Wallace

Behav Brain Res. Author manuscript; available in PMC 2019 Feb 15.

Published in final edited form as: Behav Brain Res. 2018 Feb 15; 338: 76–87. Published online 2017 Oct 14. doi: 10.1016/j.bbr.2017.10.012

PMCID:: PMC5683898

Article PDF–4.8M

Select item 473838611.

Class III myosins shape the auditory hair bundles by limiting microvilli and stereocilia growth

Andrea Lelli, Vincent Michel, Jacques Boutet de Monvel, Matteo Cortese, Montserrat Bosch-Grau, Asadollah Aghaie, Isabelle Perfettini, Typhaine Dupont, Paul Avan, Aziz El-Amraoui, Christine Petit

J Cell Biol. 2016 Jan 18; 212(2): 231–244. doi: 10.1083/jcb.201509017

PMCID:: PMC4738386

Article PDF–3.2M

Select item 399654212.

Novel skin phenotypes revealed by a genome-wide mouse reverse genetic screen

Kifayathullah Liakath-Ali, Valerie E. Vancollie, Emma Heath, Damian P. Smedley, Jeanne Estabel, David Sunter, Tia DiTommaso, Jacqueline K. White, Ramiro Ramirez-Solis, Ian Smyth, Karen P. Steel, Fiona M. Watt

Nat Commun. 2014 Apr 11; 5: 3540. Published online 2014 Apr 11. doi: 10.1038/ncomms4540

PMCID:: PMC3996542

Article PDF–4.0M

Select item 399285613.

Intestinal brush border assembly driven by protocadherin-based intermicrovillar adhesion

Scott W. Crawley, David A. Shifrin, Jr., Nathan E. Grega-Larson, Russell E. McConnell, Andrew E. Benesh, Suli Mao, Yuxi Zheng, Qing Yin Zheng, Ki Taek Nam, Bryan A. Millis, Bechara Kachar, Matthew J. Tyska

Cell. Author manuscript; available in PMC 2015 Apr 10.

Published in final edited form as: Cell. 2014 Apr 10; 157(2): 433–446. doi: 10.1016/j.cell.2014.01.067

PMCID:: PMC3992856

Article PDF–5.6M

Select item 397148314.

Usher protein functions in hair cells and photoreceptors

Dominic Cosgrove, Marisa Zallocchi

Int J Biochem Cell Biol. Author manuscript; available in PMC 2015 Jan 1.

Published in final edited form as: Int J Biochem Cell Biol. 2014 Jan; 46: 80–89. Published online 2013 Nov 12. doi: 10.1016/j.biocel.2013.11.001

PMCID:: PMC3971483

Article PDF–1.3M

Select item 371722615.

Harmonin enhances voltage-dependent facilitation of Ca_v1.3 channels and synchronous exocytosis in mouse inner hair cells

Frederick D Gregory, Tina Pangršič, Irina E Calin-Jageman, Tobias Moser, Amy Lee

J Physiol. 2013 Jul 1; 591(Pt 13): 3253–3269. Published online 2013 Apr 22. doi: 10.1113/jphysiol.2013.254367

PMCID:: PMC3717226

Article PDF–1.4M

Select item 365774416.

Rescue of hearing and vestibular function in a mouse model of human deafness

Jennifer J. Lentz, Francine M. Jodelka, Anthony J. Hinrich, Kate E. McCaffrey, Hamilton E. Farris, Mathew J. Spalitta, Nicolas G. Bazan, Dominik M. Duelli, Frank Rigo, Michelle L. Hastings

Nat Med. Author manuscript; available in PMC 2013 Sep 1.

Published in final edited form as: Nat Med. 2013 Mar; 19(3): 345–350. Published online 2013 Feb 4. doi: 10.1038/nm.3106

PMCID:: PMC3657744

Article PDF–1.1M

Select item 357241017.

A conditional knockout resource for the genome–wide study of mouse gene function

William C. Skarnes, Barry Rosen, Anthony P. West, Manousos Koutsourakis, Wendy Bushell, Vivek Iyer, Alejandro O. Mujica, Mark Thomas, Jennifer Harrow, Tony Cox, David Jackson, Jessica Severin, Patrick Biggs, Jun Fu, Michael Nefedov, Pieter J. de Jong, A. Francis Stewart, Allan Bradley

Nature. Author manuscript; available in PMC 2013 Feb 14.

Published in final edited form as: Nature. 2011 Jun 15; 474(7351): 337–342. Published online 2011 Jun 15. doi: 10.1038/nature10163

PMCID:: PMC3572410

Article PDF–1.1M

Select item 352217818.

TMHS is an Integral Component of the Mechanotransduction Machinery of Cochlear Hair Cells

Wei Xiong, Nicolas Grillet, Heather M. Elledge, Thomas F.J. Wagner, Bo Zhao, Kenneth R. Johnson, Piotr Kazmierczak, Ulrich Müller

Cell. Author manuscript; available in PMC 2013 Dec 7.

Published in final edited form as: Cell. 2012 Dec 7; 151(6): 1283–1295. doi: 10.1016/j.cell.2012.10.041

PMCID:: PMC3522178

Article PDF–2.7M

Select item 339564719.

Developmental Gene Expression Profiling along the Tonotopic Axis of the Mouse Cochlea

Eun Jin Son, Ling Wu, Heejei Yoon, Sunhee Kim, Jae Young Choi, Jinwoong Bok

PLoS One. 2012; 7(7): e40735. Published online 2012 Jul 12. doi: 10.1371/journal.pone.0040735

PMCID:: PMC3395647

Article PDF–890K

Select item 337559020.

Stereocilin connects outer-hair-cell stereocilia to one another and to the tectorial membrane

Elisabeth Verpy, Michel Leibovici, Nicolas Michalski, Richard J. Goodyear, Carine Houdon, Dominique Weil, Guy P. Richardson, Christine Petit

J Comp Neurol. Author manuscript; available in PMC 2012 Jun 15.

Published in final edited form as: J Comp Neurol. 2011 Feb 1; 519(2): 194–210. doi: 10.1002/cne.22509

PMCID:: PMC3375590

Article PDF–4.6M

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