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부신백질형성장애증 섬유모세포에서 발프로산의 항산화능

DC Field Value Language
dc.contributor.author강훈철-
dc.contributor.author장지호-
dc.date.accessioned2016-02-04T11:56:46Z-
dc.date.available2016-02-04T11:56:46Z-
dc.date.issued2015-
dc.identifier.issn1226-6884-
dc.identifier.urihttps://ir.ymlib.yonsei.ac.kr/handle/22282913/141549-
dc.description.abstractPurpose: X-linked adrenoleukodystrophy (X-ALD) is a fatal, axonal demyelinating, neurodegenerative disease, and is caused by mutations the in ABCD1 (ATP-binding cassette transporter subfamily D member 1). Oxidative damage of proteins caused by very long chain fatty acid accumulating in X-ALD, is an early event in the neuro-degenerative cascade. We evaluated valproic acid (VPA) as a possible option for oxidative damage in X-ALD. Method: We generated fibroblast of the childhood cerebral ALD from patient. We evaluated mRNA (ribonucleic acid) level of ABCD2 by real-time polymerase chain reaction, and reactive oxygen species (ROS) levels by flow cytometry. Results: VPA increased expression of ABCD2 in both control and ALD fibroblast. ABCD2 gene mRNA expression was increased 1.76 fold in normal fibroblasts, and 2.22 fold in the X-ALD fibroblasts. ROS levels were decreased in VPA treated X-ALD fibroblast, especially in treated with 1 mM of VPA. ROS levels revealed 13.7 in control fibroblast, on the other hand, 5.83 in X-ALD fibroblast treated with 1 mM of VPA. Conclusion: We propose VPA as a promising novel therapeutic approach in oxidant damage that warrants further clinical investigation in X-ALD.-
dc.description.statementOfResponsibilityopen-
dc.languageKorean-
dc.publisher대한소아신경학회-
dc.relation.isPartOfJournal of the Korean Child Neurology Society-
dc.rightsCC BY-NC-ND 2.0 KR-
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/2.0/kr/-
dc.title부신백질형성장애증 섬유모세포에서 발프로산의 항산화능-
dc.title.alternativeValproic Acid Reduces Reactive Oxygen Species in Fibroblast of X-linked Adrenoleukodystrophy-
dc.typeArticle-
dc.contributor.collegeCollege of Medicine (의과대학)-
dc.contributor.departmentDept. of Pediatrics (소아과학)-
dc.contributor.googleauthor강준원-
dc.contributor.googleauthor전철구-
dc.contributor.googleauthor장지호-
dc.contributor.googleauthor강훈철-
dc.admin.authorfalse-
dc.admin.mappingfalse-
dc.contributor.localIdA00102-
dc.contributor.localIdA03480-
dc.relation.journalcodeJ01815-
dc.identifier.urlhttp://scholar.dkyobobook.co.kr/searchDetail.laf?barcode=4010024546938#-
dc.subject.keywordAdrenoleukodystrophy-
dc.subject.keywordValproic Acid-
dc.subject.keywordReactive Oxygen Species-
dc.contributor.alternativeNameKang, Hoon Chul-
dc.contributor.alternativeNameJang, Ji Ho-
dc.contributor.affiliatedAuthorKang, Hoon Chul-
dc.contributor.affiliatedAuthorJang, Ji Ho-
dc.rights.accessRightsnot free-
dc.citation.volume23-
dc.citation.number2-
dc.citation.startPage45-
dc.citation.endPage50-
dc.identifier.bibliographicCitationJournal of the Korean Child Neurology Society, Vol.23(2) : 45-50, 2015-
dc.identifier.rimsid30715-
dc.type.rimsART-
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Physiology (생리학교실) > 1. Journal Papers

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