Neuroplastin isoform Np55 is expressed in the stereocilia of outer hair cells and required for normal outer hair cell function

Wei Zheng Zeng, Nicolas Grillet, James B. Dewey, Alix Trouillet, Jocelyn F. Krey, Peter Barr-Gillespie, John S. Oghalai, Ulrich Müller

Research output: Contribution to journalArticle

12 Scopus citations

Abstract

Neuroplastin (Nptn) is a member of the Ig superfamily and is expressed in two isoforms, Np55 and Np65. Np65 regulates synaptic transmission but the function of Np55 is unknown. In anN-ethyl-N-nitrosaurea mutagenesis screen, we have now generated a mouse line with an Nptn mutation that causes deafness. We show that Np55 is expressed in stereocilia of outer hair cells (OHCs) but not inner hair cells and affects interactions of stereocilia with the tectorial membrane. In vivo vibrometry demonstrates that cochlear amplification is absent in Nptn mutant mice, which is consistent with the failure of OHC stereocilia to maintain stable interactions with the tectorial membrane. Hair bundles show morphological defects as the mutant mice age and while mechanotransduction currents can be evoked in early postnatal hair cells, cochlea microphonics recordings indicate that mechanon transduction is affected as the mutant mice age. We thus conclude that differential splicing leads to functional diversification of Nptn, where Np55 is essential for OHC function, while Np65 is implicated in the regulation of synaptic function.

Original languageEnglish (US)
Pages (from-to)9201-9216
Number of pages16
JournalJournal of Neuroscience
Volume36
Issue number35
DOIs
Publication statusPublished - Aug 31 2016

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Keywords

  • Amplification
  • Deafness
  • Hair cells
  • Inner ear
  • Neuroplastin
  • Tectorial membrane

ASJC Scopus subject areas

  • Neuroscience(all)
  • Medicine(all)

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