The susceptibility of cochlear outer hair cells to cyclodextrin is not related to their electromotile activity

The susceptibility of cochlear outer hair cells to cyclodextrin is not related to their electromotile activity

Abstract Niemann-Pick Type C1 (NPC1) disease is a fatal neurovisceral disorder caused by dysfunction of NPC1 protein, which plays a role in intracellular cholesterol trafficking. The cholesterol-chelating agent, 2-hydroxypropyl-β-cyclodextrin (HPβCD), is currently undergoing clinical trials for trea...

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Bibliographic Details
Main Authors: Yingjie Zhou, Satoe Takahashi, Kazuaki Homma, Chongwen Duan, Jason Zheng, Mary Ann Cheatham, Jing Zheng
Format: Article
Language:English
Published: BMC 2018-09-01
Series:Acta Neuropathologica Communications
Subjects:
Prestin
Niemann-pick type C1
HPβCD
Cholesterol
Salicylate
Electromotility
Online Access:http://link.springer.com/article/10.1186/s40478-018-0599-9

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