281 275

Cited 1 times in

Exogenous Addition of 25-Hydroxycholesterol Reduces Level of Very Long-Chain Fatty Acids in X-Linked Adrenoleukodystrophy

Authors
 Jiho Jang  ;  Jung Wuk Lee  ;  Jiho Song  ;  Dong‐Wook Kim  ;  Kyung Hoon Min 
Citation
 ChemistryOpen, Vol.8(2) : 188-191, 2019 
Journal Title
CHEMISTRYOPEN
Issue Date
2019
Keywords
25-hydroxycholesterol ; X-linked adrenoleukodystrophy ; derivatives ; liver X receptor ; very long chain fatty acids
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 oligodendrocytes 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 ELOVL1 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.
Files in This Item:
T201903960.pdf Download
DOI
10.1002/open.201800281
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Physiology (생리학교실) > 1. Journal Papers
Yonsei Authors
Kim, Dong Wook(김동욱) ORCID logo https://orcid.org/0000-0002-5025-1532
Jang, Ji Ho(장지호) ORCID logo https://orcid.org/0000-0001-5551-3514
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/173119
사서에게 알리기
  feedback

qrcode

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.

Browse

Links