Cited 13 times in
ABCD2 Is a Direct Target of β-Catenin and TCF-4: Implications for X-Linked Adrenoleukodystrophy Therapy
DC Field | Value | Language |
---|---|---|
dc.contributor.author | 김동욱 | - |
dc.contributor.author | 김한수 | - |
dc.contributor.author | 박철용 | - |
dc.contributor.author | 유정은 | - |
dc.contributor.author | 이동진 | - |
dc.contributor.author | 이재석 | - |
dc.contributor.author | 이현지 | - |
dc.contributor.author | 장지호 | - |
dc.date.accessioned | 2014-12-18T09:26:07Z | - |
dc.date.available | 2014-12-18T09:26:07Z | - |
dc.date.issued | 2013 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/88164 | - |
dc.description.abstract | X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder caused by mutations in the ABCD1 gene that encodes the peroxisomal ATP-binding cassette (ABC) transporter subfamily D member 1 protein (ABCD1), which is referred to as the adrenoleukodystrophy protein (ALDP). Induction of the ABCD2 gene, the closest homolog of ABCD1, has been mentioned as a possible therapeutic option for the defective ABCD1 protein in X-ALD. However, little is known about the transcriptional regulation of ABCD2 gene expression. Here, through in silico analysis, we found two putative TCF-4 binding elements between nucleotide positions −360 and −260 of the promoter region of the ABCD2 gene. The transcriptional activity of the ABCD2 promoter was strongly increased by ectopic expression of β-catenin and TCF-4. In addition, mutation of either or both TCF-4 binding elements by site-directed mutagenesis decreased promoter activity. This was further validated by the finding that β-catenin and the promoter of the ABCD2 gene were pulled down with a β-catenin antibody in a chromatin immunoprecipitation assay. Moreover, real-time PCR analysis revealed that β-catenin and TCF-4 increased mRNA levels of ABCD2 in both a hepatocellular carcinoma cell line and primary fibroblasts from an X-ALD patient. Interestingly, we found that the levels of very long chain fatty acids were decreased by ectopic expression of ABCD2-GFP as well as β-catenin and TCF-4. Taken together, our results demonstrate for the first time the direct regulation of ABCD2 by β-catenin and TCF-4. | - |
dc.description.statementOfResponsibility | open | - |
dc.relation.isPartOf | PLOS ONE | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/2.0/kr/ | - |
dc.subject.MESH | ATP Binding Cassette Transporter, Sub-Family D | - |
dc.subject.MESH | ATP-Binding Cassette Transporters/genetics* | - |
dc.subject.MESH | Adrenoleukodystrophy/genetics* | - |
dc.subject.MESH | Adrenoleukodystrophy/pathology | - |
dc.subject.MESH | Adrenoleukodystrophy/therapy* | - |
dc.subject.MESH | Base Sequence | - |
dc.subject.MESH | Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/metabolism* | - |
dc.subject.MESH | Binding Sites/genetics | - |
dc.subject.MESH | Fatty Acids/metabolism | - |
dc.subject.MESH | Fibroblasts/metabolism | - |
dc.subject.MESH | Fibroblasts/pathology | - |
dc.subject.MESH | Gene Silencing | - |
dc.subject.MESH | Green Fluorescent Proteins/metabolism | - |
dc.subject.MESH | Hep G2 Cells | - |
dc.subject.MESH | Humans | - |
dc.subject.MESH | Molecular Sequence Data | - |
dc.subject.MESH | Molecular Targeted Therapy* | - |
dc.subject.MESH | Promoter Regions, Genetic/genetics | - |
dc.subject.MESH | RNA, Messenger/genetics | - |
dc.subject.MESH | RNA, Messenger/metabolism | - |
dc.subject.MESH | Recombinant Fusion Proteins/metabolism | - |
dc.subject.MESH | Transcription Factor 4 | - |
dc.subject.MESH | Transcription Factors/metabolism* | - |
dc.subject.MESH | Transcription, Genetic | - |
dc.subject.MESH | Transcriptional Activation/genetics | - |
dc.subject.MESH | Up-Regulation/genetics | - |
dc.subject.MESH | beta Catenin/metabolism* | - |
dc.title | ABCD2 Is a Direct Target of β-Catenin and TCF-4: Implications for X-Linked Adrenoleukodystrophy Therapy | - |
dc.type | Article | - |
dc.contributor.college | College of Medicine (의과대학) | - |
dc.contributor.department | Dept. of Laboratory Medicine (진단검사의학) | - |
dc.contributor.googleauthor | Chul-Yong Park | - |
dc.contributor.googleauthor | Han-Soo Kim | - |
dc.contributor.googleauthor | Jiho Jang | - |
dc.contributor.googleauthor | Hyunji Lee | - |
dc.contributor.googleauthor | Jae Souk Lee | - |
dc.contributor.googleauthor | Jeong-Eun Yoo | - |
dc.contributor.googleauthor | Dongjin R. Lee | - |
dc.contributor.googleauthor | Dong-Wook Kim | - |
dc.identifier.doi | 10.1371/journal.pone.0056242 | - |
dc.admin.author | false | - |
dc.admin.mapping | false | - |
dc.contributor.localId | A01100 | - |
dc.contributor.localId | A01719 | - |
dc.contributor.localId | A02734 | - |
dc.contributor.localId | A03074 | - |
dc.contributor.localId | A03299 | - |
dc.contributor.localId | A03480 | - |
dc.contributor.localId | A02505 | - |
dc.contributor.localId | A00406 | - |
dc.relation.journalcode | J02540 | - |
dc.identifier.eissn | 1932-6203 | - |
dc.identifier.pmid | 23437103 | - |
dc.subject.keyword | ATP Binding Cassette Transporter, Sub-Family D | - |
dc.subject.keyword | ATP-Binding Cassette Transporters/genetics* | - |
dc.subject.keyword | Adrenoleukodystrophy/genetics* | - |
dc.subject.keyword | Adrenoleukodystrophy/pathology | - |
dc.subject.keyword | Adrenoleukodystrophy/therapy* | - |
dc.subject.keyword | Base Sequence | - |
dc.subject.keyword | Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/metabolism* | - |
dc.subject.keyword | Binding Sites/genetics | - |
dc.subject.keyword | Fatty Acids/metabolism | - |
dc.subject.keyword | Fibroblasts/metabolism | - |
dc.subject.keyword | Fibroblasts/pathology | - |
dc.subject.keyword | Gene Silencing | - |
dc.subject.keyword | Green Fluorescent Proteins/metabolism | - |
dc.subject.keyword | Hep G2 Cells | - |
dc.subject.keyword | Humans | - |
dc.subject.keyword | Molecular Sequence Data | - |
dc.subject.keyword | Molecular Targeted Therapy* | - |
dc.subject.keyword | Promoter Regions, Genetic/genetics | - |
dc.subject.keyword | RNA, Messenger/genetics | - |
dc.subject.keyword | RNA, Messenger/metabolism | - |
dc.subject.keyword | Recombinant Fusion Proteins/metabolism | - |
dc.subject.keyword | Transcription Factor 4 | - |
dc.subject.keyword | Transcription Factors/metabolism* | - |
dc.subject.keyword | Transcription, Genetic | - |
dc.subject.keyword | Transcriptional Activation/genetics | - |
dc.subject.keyword | Up-Regulation/genetics | - |
dc.subject.keyword | beta Catenin/metabolism* | - |
dc.contributor.alternativeName | Kim, Dong Wook | - |
dc.contributor.alternativeName | Kim, Han Soo | - |
dc.contributor.alternativeName | Park, Chul Yong | - |
dc.contributor.alternativeName | Yoo, Jeong Eun | - |
dc.contributor.alternativeName | Lee, Dongjin R. | - |
dc.contributor.alternativeName | Lee, Jae Souk | - |
dc.contributor.alternativeName | Lee, Hyun Ji | - |
dc.contributor.alternativeName | Jang, Ji Ho | - |
dc.contributor.affiliatedAuthor | Kim, Han Soo | - |
dc.contributor.affiliatedAuthor | Park, Chul Yong | - |
dc.contributor.affiliatedAuthor | Lee, Dongjin R. | - |
dc.contributor.affiliatedAuthor | Lee, Jae Souk | - |
dc.contributor.affiliatedAuthor | Lee, Hyun Ji | - |
dc.contributor.affiliatedAuthor | Jang, Ji Ho | - |
dc.contributor.affiliatedAuthor | Yoo, Jeong Eun | - |
dc.contributor.affiliatedAuthor | Kim, Dong Wook | - |
dc.rights.accessRights | free | - |
dc.citation.volume | 8 | - |
dc.citation.number | 2 | - |
dc.citation.startPage | e56242 | - |
dc.identifier.bibliographicCitation | PLOS ONE, Vol.8(2) : e56242, 2013 | - |
dc.identifier.rimsid | 33101 | - |
dc.type.rims | ART | - |
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