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

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dc.contributor.author장지호-
dc.contributor.author김동욱-
dc.date.accessioned2019-12-18T00:35:13Z-
dc.date.available2019-12-18T00:35:13Z-
dc.date.issued2019-
dc.identifier.urihttps://ir.ymlib.yonsei.ac.kr/handle/22282913/173119-
dc.description.abstractX-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.-
dc.description.statementOfResponsibilityopen-
dc.formatapplication/pdf-
dc.languageEnglish-
dc.publisherWiley-VCH Verlag-
dc.relation.isPartOfChemistryOpen-
dc.rightsCC BY-NC-ND 2.0 KR-
dc.titleExogenous Addition of 25-Hydroxycholesterol Reduces Level of Very Long-Chain Fatty Acids in X-Linked Adrenoleukodystrophy-
dc.typeArticle-
dc.contributor.collegeCollege of Medicine (의과대학)-
dc.contributor.departmentDept. of Physiology (생리학교실)-
dc.contributor.googleauthorJiho Jang-
dc.contributor.googleauthorJung Wuk Lee-
dc.contributor.googleauthorJiho Song-
dc.contributor.googleauthorDong‐Wook Kim-
dc.contributor.googleauthorKyung Hoon Min-
dc.identifier.doi10.1002/open.201800281-
dc.contributor.localIdA03480-
dc.relation.journalcodeJ03714-
dc.identifier.eissn2191-1363-
dc.identifier.pmid30788208-
dc.subject.keyword25-hydroxycholesterol-
dc.subject.keywordX-linked adrenoleukodystrophy-
dc.subject.keywordderivatives-
dc.subject.keywordliver X receptor-
dc.subject.keywordvery long chain fatty acids-
dc.contributor.alternativeNameJang, Ji Ho-
dc.contributor.affiliatedAuthor장지호-
dc.citation.volume8-
dc.citation.number2-
dc.citation.startPage188-
dc.citation.endPage191-
dc.identifier.bibliographicCitationChemistryOpen, Vol.8(2) : 188-191, 2019-
dc.identifier.rimsid64302-
dc.type.rimsART-
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Physiology (생리학교실) > 1. Journal Papers

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