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

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 treatment of...

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Detalles Bibliográficos
Publicado en:Acta Neuropathol Commun
Main Authors: Zhou, Yingjie, Takahashi, Satoe, Homma, Kazuaki, Duan, Chongwen, Zheng, Jason, Cheatham, Mary Ann, Zheng, Jing
Formato: Artigo
Idioma:Inglês
Publicado: BioMed Central 2018
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Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC6151916/
https://ncbi.nlm.nih.gov/pubmed/30249300
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s40478-018-0599-9
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