期刊
JOURNAL OF NEUROSCIENCE
卷 36, 期 35, 页码 9201-9216出版社
SOC NEUROSCIENCE
DOI: 10.1523/JNEUROSCI.0093-16.2016
关键词
amplification; deafness; hair cells; inner ear; neuroplastin; tectorial membrane
资金
- National Institute on Deafness and Other Communication Disorders [DC005965, DC007704, DC014713, DC014450, DC013774, DC010363]
- Dorris Neuroscience Center
- Skaggs Institute for Chemical Biology
- Dean's Postdoctoral Fellowship at the Stanford University School of Medicine
- George E. Hewitt Foundation for Medical Research
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 mechanontransduction 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.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据