The ubiquitin-conjugating enzyme UbcH6 regulates the transcriptional repression activity of the SCA1 gene product ataxin-1
DC Field | Value | Language |
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dc.contributor.author | Lee, Soyeon | - |
dc.contributor.author | Hong, Sunghoi | - |
dc.contributor.author | Kang, Seongman | - |
dc.date.accessioned | 2021-09-09T05:10:37Z | - |
dc.date.available | 2021-09-09T05:10:37Z | - |
dc.date.created | 2021-06-10 | - |
dc.date.issued | 2008-08-08 | - |
dc.identifier.issn | 0006-291X | - |
dc.identifier.uri | https://scholar.korea.ac.kr/handle/2021.sw.korea/122866 | - |
dc.description.abstract | Spinocerebellar ataxia type 1 (SCA1) is an autosomal-dominant neurodegenerative disorder characterized by ataxia and progressive motor deterioration. SCA1 is caused by expansion of the polyglutamine tract in the SCA1 gene product, ataxin-1. We previously reported that the E2 ubiquitin-conjugating enzyme UbcH6 interacts with and ubiquitinates the ataxin-1 proteins as an E2-substrate cognate pair in the ubiquitin-proteasome system. In the present study, we further investigated whether the function of ataxin-1 is associated with UbcH6 and found that UbcH6 regulates the transcriptional repression activity of ataxin-1. The overexpression of UbcH6 reduced the transcriptional repression activity of ataxin-1. Interestingly, ataxin-1(30Q) was more affected by the presence of UbcH6 than ataxin-1(82Q), implying that the length of the polyglutamine tract in ataxin-1 might be involved in determining the stability of ataxin-1. The half-life of ataxin-1(82Q) was longer than that of ataxin-1(30Q) in the presence of UbcH6. shRNAs targeting UbcH6 enhanced the transcriptional repression activity of ataxin-1. In addition, the overexpression of UbcH6 reduced the formation of ataxin-1 aggregates. Our studies demonstrate that UbcH6 modulates the transcriptional repression activity of ataxin-1 by modulating the degradation of ataxin-1, suggesting that UbcH6 may have some therapeutic potential in the treatment of SCA1. (c) 2008 Elsevier Inc. All rights reserved. | - |
dc.language | English | - |
dc.language.iso | en | - |
dc.publisher | ACADEMIC PRESS INC ELSEVIER SCIENCE | - |
dc.subject | MUTANT ATAXIN-1 | - |
dc.subject | AXH DOMAIN | - |
dc.subject | PROTEIN | - |
dc.subject | MICE | - |
dc.subject | NEURODEGENERATION | - |
dc.subject | INTERACTS | - |
dc.subject | LOCALIZATION | - |
dc.subject | AGGREGATION | - |
dc.subject | SUPPRESSES | - |
dc.subject | EXPANSION | - |
dc.title | The ubiquitin-conjugating enzyme UbcH6 regulates the transcriptional repression activity of the SCA1 gene product ataxin-1 | - |
dc.type | Article | - |
dc.contributor.affiliatedAuthor | Hong, Sunghoi | - |
dc.contributor.affiliatedAuthor | Kang, Seongman | - |
dc.identifier.doi | 10.1016/j.bbrc.2008.05.125 | - |
dc.identifier.scopusid | 2-s2.0-46149116525 | - |
dc.identifier.wosid | 000257419500041 | - |
dc.identifier.bibliographicCitation | BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, v.372, no.4, pp.735 - 740 | - |
dc.relation.isPartOf | BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | - |
dc.citation.title | BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | - |
dc.citation.volume | 372 | - |
dc.citation.number | 4 | - |
dc.citation.startPage | 735 | - |
dc.citation.endPage | 740 | - |
dc.type.rims | ART | - |
dc.type.docType | Article | - |
dc.description.journalClass | 1 | - |
dc.description.journalRegisteredClass | scie | - |
dc.description.journalRegisteredClass | scopus | - |
dc.relation.journalResearchArea | Biochemistry & Molecular Biology | - |
dc.relation.journalResearchArea | Biophysics | - |
dc.relation.journalWebOfScienceCategory | Biochemistry & Molecular Biology | - |
dc.relation.journalWebOfScienceCategory | Biophysics | - |
dc.subject.keywordPlus | MUTANT ATAXIN-1 | - |
dc.subject.keywordPlus | AXH DOMAIN | - |
dc.subject.keywordPlus | PROTEIN | - |
dc.subject.keywordPlus | MICE | - |
dc.subject.keywordPlus | NEURODEGENERATION | - |
dc.subject.keywordPlus | INTERACTS | - |
dc.subject.keywordPlus | LOCALIZATION | - |
dc.subject.keywordPlus | AGGREGATION | - |
dc.subject.keywordPlus | SUPPRESSES | - |
dc.subject.keywordPlus | EXPANSION | - |
dc.subject.keywordAuthor | ataxin-1 | - |
dc.subject.keywordAuthor | UbcH6 | - |
dc.subject.keywordAuthor | transcriptional regulation | - |
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