| アイテムタイプ |
文献 / Documents(1) |
| 公開日 |
2025-07-31 |
| アクセス権 |
|
|
アクセス権 |
embargoed access |
|
アクセス権URI |
http://purl.org/coar/access_right/c_f1cf |
| 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 |
| 出版社版DOI |
|
|
|
関連識別子 |
https://doi.org/10.1016/j.bbalip.2025.159670 |
|
|
関連名称 |
10.1016/j.bbalip.2025.159670 |
| 出版タイプ |
|
|
出版タイプ |
AM |
|
出版タイプResource |
http://purl.org/coar/version/c_ab4af688f83e57aa |
| タイトル |
|
|
タイトル |
Different effects of Lorenzo's oil components against very long-chain fatty acid-induced endoplasmic reticulum stress in peroxisome-deficient CHO cells |
| 著者 |
Ali, Hanif
Yamanishi, Mone
Hasi, Rumana Yesmin
Islam, Majidul
濱田, 良真
三宅, 雅人
親泊, 政一
Kiyokage, Emi
Toida, Kazunori
川上, 竜巳
(中橋)粟飯原, 睦美
田中, 保
|
| 抄録 |
|
|
内容記述 |
Adrenoleukodystrophy (ALD) is an X-linked peroxisomal disorder caused by mutations in the ABCD1 gene, leading to the accumulation of very long-chain fatty acids (VLCFAs). The accumulation of saturated VLCFAs, such as C24:0 and C26:0, is believed to impair myelination. A mixture of C18:1 (oleic acid) and C22:1 (erucic acid), known as Lorenzo’s oil, has been used to reduce these saturated VLCFAs. However, despite lowering saturated VLCFA levels, Lorenzo’s oil proved ineffective in preventing neurological symptoms. Previously, we found that VLCFA-induced apoptosis is prevented by C18:1 supplementation in peroxisome-deficient Chinese Hamster Overy (CHO) cells. In this study, we investigated the mechanism underlying the rescue effect of C18:1 and examined the effect of C22:1, another component of Lorenzo’s oil. Supplementation with C18:1 completely rescued the cells from VLCFA-induced apoptosis. In contrast, C22:1 enhanced VLCFA cytotoxicity and diminished the protective effect of C18:1. We found that VLCFA-induced apoptosis is mediated via the endoplasmic reticulum (ER) stress response possibly by disruption of ER structure, whereas C18:1 attenuated this ER stress. Quantitative lipidomics revealed that VLCFAs were predominantly incorporated into phosphatidylcholine (PC), accompanied by a significant reduction in PC species containing C18:1. Among these, PC 36:2 (18:1/18:1) showed a pattern of change that correlated with cellular viability. These results indicate that C18:1, but not C22:1, protects peroxisome-deficient CHO cells by ameliorating the ER stress response, likely through improving ER structure distorted by VLCFA accumulation. |
| キーワード |
|
|
主題 |
Peroxisome disease |
| キーワード |
|
|
主題 |
Very long-chain fatty acids |
| キーワード |
|
|
主題 |
Oleic acid |
| キーワード |
|
|
主題 |
Endoplasmic reticulum stress |
| キーワード |
|
|
主題 |
Apoptosis |
| 書誌情報 |
en : Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids
発行日 2025-07-27
|
| 収録物ID |
|
|
収録物識別子タイプ |
PISSN |
|
収録物識別子 |
13881981 |
| 収録物ID |
|
|
収録物識別子タイプ |
EISSN |
|
収録物識別子 |
18792618 |
| 収録物ID |
|
|
収録物識別子タイプ |
NCID |
|
収録物識別子 |
AA11522965 |
| 収録物ID |
|
|
収録物識別子タイプ |
NCID |
|
収録物識別子 |
AA11300409 |
| 出版者 |
|
|
出版者 |
Elsevier |
| 権利情報 |
|
|
権利情報 |
© 2025. This manuscript version is made available under the CC-BY-NC-ND 4.0 license https://creativecommons.org/licenses/by-nc-nd/4.0/ |
| EID |
|
|
識別子 |
446042 |
| 言語 |
|
|
言語 |
eng |
bbalip.pdf (571 KB)
