Cholesterol-dependent increases in glucosylceramide synthase activity in Niemann-Pick disease type C model cells: Abnormal trafficking of endogenously formed ceramide metabolites by inhibition of the enzyme
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- 作者：Naohiro Hashimoto1 ; Ikiru Matsumoto1 ; Hiromasa Takahashi ; Hitomi Ashikawa ; Hiroyuki Nakamura ; nakahiro@faculty.chiba-u.jp" class="auth_mail" title="E-mail the corresponding author ; Toshihiko Murayama
- 关键词：Niemann-Pick disease type C ; Cholesterol ; Glucosylceramide synthase ; Sphingomyelin ; Vesicular trafficking
- 刊名：Neuropharmacology
- 出版年：2016
- 出版时间：November 2016
- 年：2016
- 卷：110
- 期：part_PA
- 页码：458-469
- 全文大小：1949 K
文摘
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In NPC cells, GluCer synthase activity was increased depending on cholesterol.
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NPC cells showed an abnormal vesicular trafficking of ceramide metabolites.
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NB-DNJ treatment enhanced the abnormal trafficking with cholesterol increase.
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GlcCer (increase/decrease) and cholesterol regulate the trafficking in NPC cells.
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For taking NB-DNJ, cellular cholesterol/vesicular trafficking should be monitored.