Essential role of retinoblastoma protein in mammalian hair cell development and hearing.

Proceedings of the National Academy of Sciences of the United States of America
Cyrille SageZheng-Yi Chen

Abstract

The retinoblastoma protein pRb is required for cell-cycle exit of embryonic mammalian hair cells but not for their early differentiation. However, its role in postnatal hair cells is unknown. To study the function of pRb in mature animals, we created a new conditional mouse model, with the Rb gene deleted primarily in the inner ear. Progeny survive up to 6 months. During early postnatal development, pRb(-/-) hair cells continue to divide and can transduce mechanical stimuli. However, adult pRb(-/-) mice exhibit profound hearing loss due to progressive degeneration of the organ of Corti. We show that pRb is required for the full maturation of cochlear outer hair cells, likely in a gene-specific manner, and is also essential for their survival. In addition, lack of pRb results in cell division in postnatal auditory supporting cells. In contrast, many pRb(-/-) vestibular hair cells survive and continue to divide in adult mice. Significantly, adult pRb(-/-) vestibular hair cells are functional, and pRb(-/-) mice maintain partial vestibular function. Therefore, the functional adult vestibular pRb(-/-) hair cells, derived from proliferation of postnatal hair cells, are largely integrated into vestibular pathways. This study reveals e...Continue Reading

References

Aug 8, 1998·Genes & Development·N Dyson
Feb 18, 1999·The Journal of Comparative Neurology·A GlosterF D Miller
Mar 18, 1999·Nature Genetics·P J LanfordM W Kelley
Mar 31, 1999·Proceedings of the National Academy of Sciences of the United States of America·H LöwenheimH P Zenner
Jun 12, 1999·Science·N A BerminghamH Y Zoghbi
Aug 24, 1999·Developmental Biology·Y Raz, M W Kelley
Jan 12, 2000·Oncogene·M M Lipinski, T Jacks
Apr 13, 2000·Proceedings of the National Academy of Sciences of the United States of America·T KharkovetsT J Jentsch
Mar 22, 2001·Proceedings of the National Academy of Sciences of the United States of America·A E KiernanM Hrabé de Angelis
Feb 19, 2002·Nature Genetics·Sarah VreugdeKaren P Steel
Oct 31, 2002·Development·Je-Hwang RyuJang-Soo Chun
Nov 8, 2002·Development·Laura S Gammill, Marianne Bronner-Fraser
Dec 3, 2002·Nature Reviews. Cancer·Marie Classon, Ed Harlow
Sep 16, 2003·Nature Neuroscience·Gwénaëlle S G Géléoc, Jeffrey R Holt
Jun 23, 2004·Brain Research. Molecular Brain Research·Xudong WuJian Zuo
Aug 27, 2004·The Journal of Comparative Neurology·Kristen Radde-GallwitzPing Chen

❮ Previous
Next ❯

Citations

Jan 23, 2008·HNO·S Pfannenstiel, M Praetorius
Oct 31, 2009·Mammalian Genome : Official Journal of the International Mammalian Genome Society·Lilach M Friedman, Karen B Avraham
May 20, 2009·Journal of the Association for Research in Otolaryngology : JARO·Julián TarandaA Belén Elgoyhen
Apr 8, 2011·Journal of the Association for Research in Otolaryngology : JARO·Mark A ParkerAlbert S B Edge
Apr 25, 2012·Journal of the Association for Research in Otolaryngology : JARO·Brandon C CoxJian Zuo
Nov 21, 1993·Biochimica Et Biophysica Acta·F López-GarcíaJ C Gómez-Fernández
Jun 24, 1994·Chemistry and Physics of Lipids·A Kõiv, P K Kinnunen
Feb 19, 1992·Biochimica Et Biophysica Acta·C el-MoatassimJ C Mani
Aug 14, 1992·Biochimica Et Biophysica Acta·S Cockcroft
Dec 21, 1993·Biochimica Et Biophysica Acta·Y A Hannun, C M Linardic
Jun 9, 1992·Biochimica Et Biophysica Acta·I I MoraruD K Das
Jul 9, 1999·Biochimica Et Biophysica Acta·R GhidoniA Giuliani
Jul 30, 1999·Biochimica Et Biophysica Acta·M E Venable, L M Obeid
May 28, 2009·Nature Neuroscience·John V Brigande, Stefan Heller
Jan 8, 2008·Proceedings of the National Academy of Sciences of the United States of America·Thomas WeberJian Zuo
May 30, 2009·Proceedings of the National Academy of Sciences of the United States of America·Janneke M StapelbroekRoderick H J Houwen
May 7, 2009·Proceedings of the National Academy of Sciences of the United States of America·Lilach M FriedmanKaren B Avraham
Aug 27, 2009·Proceedings of the National Academy of Sciences of the United States of America·Seth D BermanJacqueline A Lees
Sep 18, 2008·Current Opinion in Otolaryngology & Head and Neck Surgery·Maria Sol ColladoJeffrey T Corwin
May 18, 2011·The Journal of Clinical Investigation·Masato FujiokaAlbert S B Edge
Apr 22, 2010·Experimental Biology and Medicine·Andrew K Groves
Aug 3, 2012·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Zhiyong LiuJian Zuo
Jun 17, 2011·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Sonia M Rocha-SanchezEdward J Walsh
Apr 30, 2010·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Yiling YuJian Zuo
Feb 9, 2012·International Journal of Molecular Sciences·Nicola A Maiorano, Robert Hindges
Mar 20, 2012·Yi chuan = Hereditas·Fei XuDuan Ma
Apr 8, 2014·Proceedings of the National Academy of Sciences of the United States of America·Linjia JiangTatjana Piotrowski
Nov 24, 2012·British Medical Bulletin·Marcelo N Rivolta
Sep 21, 2013·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Mingqian HuangZheng-Yi Chen

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