IDEAS home Printed from https://ideas.repec.org/a/nat/natcom/v10y2019i1d10.1038_s41467-019-09074-4.html
   My bibliography  Save this article

LMO7 deficiency reveals the significance of the cuticular plate for hearing function

Author

Listed:
  • Ting-Ting Du

    (University of Virginia)

  • James B. Dewey

    (University of Southern California)

  • Elizabeth L. Wagner

    (University of Virginia)

  • Runjia Cui

    (National Institute of Health)

  • Jinho Heo

    (University of Virginia)

  • Jeong-Jin Park

    (University of Virginia)

  • Shimon P. Francis

    (University of Virginia)

  • Edward Perez-Reyes

    (University of Virginia)

  • Stacey J. Guillot

    (University of Virginia)

  • Nicholas E. Sherman

    (University of Virginia)

  • Wenhao Xu

    (University of Virginia)

  • John S Oghalai

    (University of Southern California)

  • Bechara Kachar

    (National Institute of Health)

  • Jung-Bum Shin

    (University of Virginia)

Abstract

Sensory hair cells, the mechanoreceptors of the auditory and vestibular systems, harbor two specialized elaborations of the apical surface, the hair bundle and the cuticular plate. In contrast to the extensively studied mechanosensory hair bundle, the cuticular plate is not as well understood. It is believed to provide a rigid foundation for stereocilia motion, but specifics about its function, especially the significance of its integrity for long-term maintenance of hair cell mechanotransduction, are not known. We discovered that a hair cell protein called LIM only protein 7 (LMO7) is specifically localized in the cuticular plate and the cell junction. Lmo7 KO mice suffer multiple cuticular plate deficiencies, including reduced filamentous actin density and abnormal stereociliar rootlets. In addition to the cuticular plate defects, older Lmo7 KO mice develop abnormalities in inner hair cell stereocilia. Together, these defects affect cochlear tuning and sensitivity and give rise to late-onset progressive hearing loss.

Suggested Citation

  • Ting-Ting Du & James B. Dewey & Elizabeth L. Wagner & Runjia Cui & Jinho Heo & Jeong-Jin Park & Shimon P. Francis & Edward Perez-Reyes & Stacey J. Guillot & Nicholas E. Sherman & Wenhao Xu & John S Og, 2019. "LMO7 deficiency reveals the significance of the cuticular plate for hearing function," Nature Communications, Nature, vol. 10(1), pages 1-15, December.
    • Handle: RePEc:nat:natcom:v:10:y:2019:i:1:d:10.1038_s41467-019-09074-4
    DOI: 10.1038/s41467-019-09074-4
    as

    Download full text from publisher

    File URL: https://www.nature.com/articles/s41467-019-09074-4
    File Function: Abstract
    Download Restriction: no
    File URL: https://libkey.io/10.1038/s41467-019-09074-4?utm_source=ideas
    LibKey link: if access is restricted and if your library uses this service, LibKey will redirect you to where you can use your library subscription to access this item
    ---><---

    More about this item

    Statistics

    Access and download statistics

    Corrections

    All material on this site has been provided by the respective publishers and authors. You can help correct errors and omissions. When requesting a correction, please mention this item's handle: RePEc:nat:natcom:v:10:y:2019:i:1:d:10.1038_s41467-019-09074-4. See general information about how to correct material in RePEc.

    If you have authored this item and are not yet registered with RePEc, we encourage you to do it here. This allows to link your profile to this item. It also allows you to accept potential citations to this item that we are uncertain about.

    We have no bibliographic references for this item. You can help adding them by using this form .

    If you know of missing items citing this one, you can help us creating those links by adding the relevant references in the same way as above, for each refering item. If you are a registered author of this item, you may also want to check the "citations" tab in your RePEc Author Service profile, as there may be some citations waiting for confirmation.

    For technical questions regarding this item, or to correct its authors, title, abstract, bibliographic or download information, contact: Sonal Shukla or Springer Nature Abstracting and Indexing (email available below). General contact details of provider: http://www.nature.com .

    Please note that corrections may take a couple of weeks to filter through the various RePEc services.

    IDEAS is a RePEc service. RePEc uses bibliographic data supplied by the respective publishers.