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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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Principais autores: Yingjie Zhou, Satoe Takahashi, Kazuaki Homma, Chongwen Duan, Jason Zheng, Mary Ann Cheatham, Jing Zheng
פורמט: Artigo
שפה:Inglês
יצא לאור: BMC 2018-09-01
סדרה:Acta Neuropathologica Communications
נושאים:
Prestin
Niemann-pick type C1
HPβCD
Cholesterol
Salicylate
Electromotility
גישה מקוונת:http://link.springer.com/article/10.1186/s40478-018-0599-9
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