Open AccessLMO7 deficiency reveals the significance of the cuticular plate for hearing function
Author(s) -
Ting-Ting Du,
James B. Dewey,
Elizabeth Wagner,
Runjia Cui,
Jun Heo,
Jeong Jin Park,
Shimon P. Francis,
Edward PerezReyes,
Stacey J. Guillot,
Nicholas E. Sherman,
Wenhao Xu,
John S. Oghalai,
Bechara Kachar,
Jung-Bum Shin
Publication year - 2019
Publication title -
nature communications
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.559
H-Index - 365
ISSN - 2041-1723
DOI - 10.1038/s41467-019-09074-4
Subject(s) - stereocilia (inner ear) , hair cell , mechanotransduction , inner ear , biology , kinocilium , anatomy , cochlea , microbiology and biotechnology , vestibular system , neuroscience
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.