Exogenous Addition of 25-Hydroxycholesterol Reduces Level of Very Long-Chain Fatty Acids in X-Linked Adrenoleukodystrophyopen access
- Authors
- Jang, Jiho; Lee, Jung Wuk; Song, Jiho; Kim, Dong-Wook; Min, Kyung Hoon
- Issue Date
- Feb-2019
- Publisher
- WILEY-V C H VERLAG GMBH
- Keywords
- 25-hydroxycholesterol; X-linked adrenoleukodystrophy; very long chain fatty acids; liver X receptor; derivatives
- Citation
- CHEMISTRYOPEN, v.8, no.2, pp 188 - 191
- Pages
- 4
- Journal Title
- CHEMISTRYOPEN
- Volume
- 8
- Number
- 2
- Start Page
- 188
- End Page
- 191
- URI
- https://scholarworks.bwise.kr/cau/handle/2019.sw.cau/18297
- DOI
- 10.1002/open.201800281
- ISSN
- 2191-1363
2191-1363
- Abstract
- X-Linked adrenoleukodystrophy (X-ALD) is a severe metabolic disorder characterized by the accumulation of very long-chain fatty acids (VLCFAs). Recently, we demonstrated that levels of 25-hydroxycholesterol (25-HC) and cholesterol 25-hydroxylase (CH25H) were found to be elevated in X-ALD. Herein, we report that the exogenous addition of 25-HC significantly reduces C26:0 levels in X-ALD patient-derived fibroblasts and oligoden-drocytes differentiated from induced pluripotent stem cells (iPSCs) derived from X-ALD patients. Moreover, 25-HC treatment was found to down-regulate the expression of ELOVL1, a key enzyme for the synthesis of C26. In addition, activation of liver X receptor (LXR), a molecular target of endogenous 25-HC, also reduced C26:0 level. The reduction of C26:0 levels by 25-HC treatment might result, at least partially, from the decrease of ELOVLI expression as well as the activation of LXR. Our findings could provide a better understanding of the role of 25-HC in X-ALD and useful information to find therapeutic agents to treat X-ALD.
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