| Item type |
学術雑誌論文 / Journal Article(1) |
| 公開日 |
2016-08-01 |
| タイトル |
|
|
タイトル |
ACID SPHINGOMYELINASE IS ESSENTIAL FOR CHOLESTEROLREDUCING AGENTS TO POSITIVELY AFFECT ACCUMULATED FREE CHOLESTEROL IN NIEMANN-PICK DISEASE TYPE C FIBROBLASTS |
|
言語 |
en |
| 言語 |
|
|
言語 |
eng |
| 主題 |
|
|
言語 |
en |
|
主題Scheme |
Other |
|
主題 |
filipin staining |
| 主題 |
|
|
言語 |
en |
|
主題Scheme |
Other |
|
主題 |
Niemann-Pick disease types A and B |
| 主題 |
|
|
言語 |
en |
|
主題Scheme |
Other |
|
主題 |
Niemann-Pick disease type C |
| 主題 |
|
|
言語 |
en |
|
主題Scheme |
Other |
|
主題 |
desipramine |
| 資源タイプ |
|
|
資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
|
資源タイプ |
journal article |
| アクセス権 |
|
|
アクセス権 |
open access |
|
アクセス権URI |
http://purl.org/coar/access_right/c_abf2 |
| 作成者 |
HIRAYAMA, Masashi
NOGUCHI, Atsuko
OYAMA, Chikako
HIRAI, Daishi
TAKAHASHI, Tsutomu
|
| 内容記述 |
|
|
内容記述タイプ |
Abstract |
|
内容記述 |
Niemann-Pick disease (NP) is a group of lipid storage disorders that includes three types of diseases. While NP type A (NP-A) and NP type B (NP-B) are caused by a lysosomal acid sphingomyelinase (ASM) deficiency, NP type C (NP-C) is a neurovisceral disorder caused by a deficiency of either the NPC1 or NPC2 protein. This deficiency causes impaired intracellular lipid trafficking, leading to the abundant accumulation of free cholesterol in the lysosome. Thus, NP-C is diagnosed through a filipin test that microscopically detects intracellular free cholesterol in fibroblasts. However, the filipin test was reported to give a positive result even for NP-A and NP-B. Recently, some cholesterol-reducing agents have been reported to be useful candidate for NP-C therapies to reduce accumulated free cholesterol level. In this study, three cholesterol-reducing agents, methyl-β-cyclodextrin, histone deacetylase inhibitors, and thapsigargin, were tested to determine their efficacy in reducing the accumulated free cholesterol level in NP-A and NP-B fibroblasts and drug-induced, ASM-deficient fibroblasts. The results showed that the three agents did not decrease the accumulated free cholesterol level in these cells, showing that ASM is essential for the cholesterol-reducing agents to be effective. This study also suggests that the accumulated free cholesterol of ASM deficient fibroblasts may be originally different from that of NP-C cells. |
|
言語 |
en |
| 出版タイプ |
|
|
出版タイプ |
VoR |
|
出版タイプResource |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| 書誌情報 |
ja : 秋田医学
巻 42,
号 3/4,
p. 137-146,
発行日 2016-03-31
|
| 収録物識別子 |
|
|
収録物識別子タイプ |
ISSN |
|
収録物識別子 |
03866106 |
| 収録物識別子 |
|
|
収録物識別子タイプ |
NCID |
|
収録物識別子 |
AN00009294 |
| 出版者 |
|
|
出版者 |
秋田医学会 |
|
言語 |
ja |
akitai42-3_4(137).pdf (1.0 MB)
