{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Du TT"],"funding":["Intramural NIH HHS","NIDCD NIH HHS","NIGMS NIH HHS","NIH HHS"],"pagination":["1117"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC6408450"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["10(1)"],"pubmed_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."],"journal":["Nature communications"],"pubmed_title":["LMO7 deficiency reveals the significance of the cuticular plate for hearing function."],"pmcid":["PMC6408450"],"funding_grant_id":["R01 DC014450","Z01 DC000002","R01 DC014254","S10 OD011966","DC013774","R01 DC013774","T32 GM139787","F31 DC017370","T32 GM008136","F32 DC016211","DC016211","DC014254","DC014450"],"pubmed_authors":["Perez-Reyes E","Kachar B","Park JJ","Wagner EL","Guillot SJ","Shin JB","Cui R","Dewey JB","Francis SP","Heo J","Du TT","Sherman NE","Xu W","Oghalai JS"],"additional_accession":[]},"is_claimable":false,"name":"LMO7 deficiency reveals the significance of the cuticular plate for hearing function.","description":"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.","dates":{"release":"2019-01-01T00:00:00Z","publication":"2019 Mar","modification":"2026-04-15T11:31:07.944Z","creation":"2019-06-06T20:58:26Z"},"accession":"S-EPMC6408450","cross_references":{"pubmed":["30850599"],"doi":["10.1038/s41467-019-09074-4"]}}