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| http://purl.uniprot.org/citations/18519031 | http://www.w3.org/2000/01/rdf-schema#comment | "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."xsd:string |
| http://purl.uniprot.org/citations/18519031 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.bbrc.2008.05.125"xsd:string |
| http://purl.uniprot.org/citations/18519031 | http://purl.uniprot.org/core/author | "Hong S."xsd:string |
| http://purl.uniprot.org/citations/18519031 | http://purl.uniprot.org/core/author | "Kang S."xsd:string |
| http://purl.uniprot.org/citations/18519031 | http://purl.uniprot.org/core/author | "Lee S."xsd:string |
| http://purl.uniprot.org/citations/18519031 | http://purl.uniprot.org/core/date | "2008"xsd:gYear |
| http://purl.uniprot.org/citations/18519031 | http://purl.uniprot.org/core/name | "Biochem Biophys Res Commun"xsd:string |
| http://purl.uniprot.org/citations/18519031 | http://purl.uniprot.org/core/pages | "735-740"xsd:string |
| http://purl.uniprot.org/citations/18519031 | http://purl.uniprot.org/core/title | "The ubiquitin-conjugating enzyme UbcH6 regulates the transcriptional repression activity of the SCA1 gene product ataxin-1."xsd:string |
| http://purl.uniprot.org/citations/18519031 | http://purl.uniprot.org/core/volume | "372"xsd:string |
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