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부신백질형성장애증 섬유모세포에서 발프로산의 항산화능
DC Field | Value | Language |
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dc.contributor.author | 강훈철 | - |
dc.contributor.author | 장지호 | - |
dc.date.accessioned | 2016-02-04T11:56:46Z | - |
dc.date.available | 2016-02-04T11:56:46Z | - |
dc.date.issued | 2015 | - |
dc.identifier.issn | 1226-6884 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/141549 | - |
dc.description.abstract | Purpose: 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.statementOfResponsibility | open | - |
dc.language | Korean | - |
dc.publisher | 대한소아신경학회 | - |
dc.relation.isPartOf | Journal of the Korean Child Neurology Society | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/2.0/kr/ | - |
dc.title | 부신백질형성장애증 섬유모세포에서 발프로산의 항산화능 | - |
dc.title.alternative | Valproic Acid Reduces Reactive Oxygen Species in Fibroblast of X-linked Adrenoleukodystrophy | - |
dc.type | Article | - |
dc.contributor.college | College of Medicine (의과대학) | - |
dc.contributor.department | Dept. of Pediatrics (소아과학) | - |
dc.contributor.googleauthor | 강준원 | - |
dc.contributor.googleauthor | 전철구 | - |
dc.contributor.googleauthor | 장지호 | - |
dc.contributor.googleauthor | 강훈철 | - |
dc.admin.author | false | - |
dc.admin.mapping | false | - |
dc.contributor.localId | A00102 | - |
dc.contributor.localId | A03480 | - |
dc.relation.journalcode | J01815 | - |
dc.identifier.url | http://scholar.dkyobobook.co.kr/searchDetail.laf?barcode=4010024546938# | - |
dc.subject.keyword | Adrenoleukodystrophy | - |
dc.subject.keyword | Valproic Acid | - |
dc.subject.keyword | Reactive Oxygen Species | - |
dc.contributor.alternativeName | Kang, Hoon Chul | - |
dc.contributor.alternativeName | Jang, Ji Ho | - |
dc.contributor.affiliatedAuthor | Kang, Hoon Chul | - |
dc.contributor.affiliatedAuthor | Jang, Ji Ho | - |
dc.rights.accessRights | not free | - |
dc.citation.volume | 23 | - |
dc.citation.number | 2 | - |
dc.citation.startPage | 45 | - |
dc.citation.endPage | 50 | - |
dc.identifier.bibliographicCitation | Journal of the Korean Child Neurology Society, Vol.23(2) : 45-50, 2015 | - |
dc.identifier.rimsid | 30715 | - |
dc.type.rims | ART | - |
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