| http://purl.uniprot.org/citations/22970133 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/22970133 | http://www.w3.org/2000/01/rdf-schema#comment | "Alterations in the ubiquitin-proteasome system (UPS) have been reported in several neurodegenerative disorders characterized by protein misfolding and aggregation, including the polylgutamine diseases. Machado-Joseph disease (MJD) or Spinocerebellar Ataxia type 3 is caused by a polyglutamine-encoding CAG expansion in the ATXN3 gene, which encodes a 42 kDa deubiquitinating enzyme (DUB), ataxin-3. We investigated ataxin-3 deubiquitinating activity and the functional relevance of ataxin-3 interactions with two proteins previously described to interact with ataxin-3, hHR23A and valosin-containing protein (VCP/p97). We confirmed ataxin-3 affinity for both hHR23A and VCP/p97. hHR23A and ataxin-3 were shown to co-localize in discrete nuclear foci, while VCP/p97 was primarily cytoplasmic. hHR23A and VCP/p97 recombinant proteins were added, separately or together, to normal and expanded ataxin-3 in in vitro deubiquitination assays to evaluate their influence on ataxin-3 activity. VCP/p97 was shown to be an activator specifically of wild-type ataxin-3, exhibiting no effect on expanded ataxin-3, In contrast, we observed no significant alterations in ataxin-3 enzyme kinetics or substrate preference in the presence of hHR23A alone or in combination with VCP. Based on our results we propose a model where ataxin-3 normally functions with its interactors to specify the cellular fate of ubiquitinated proteins."xsd:string |
| http://purl.uniprot.org/citations/22970133 | http://purl.org/dc/terms/identifier | "doi:10.1371/journal.pone.0043563"xsd:string |
| http://purl.uniprot.org/citations/22970133 | http://purl.uniprot.org/core/author | "Cortes L."xsd:string |
| http://purl.uniprot.org/citations/22970133 | http://purl.uniprot.org/core/author | "Paulson H.L."xsd:string |
| http://purl.uniprot.org/citations/22970133 | http://purl.uniprot.org/core/author | "Travis S.M."xsd:string |
| http://purl.uniprot.org/citations/22970133 | http://purl.uniprot.org/core/author | "Rego A.C."xsd:string |
| http://purl.uniprot.org/citations/22970133 | http://purl.uniprot.org/core/author | "Laco M.N."xsd:string |
| http://purl.uniprot.org/citations/22970133 | http://purl.uniprot.org/core/date | "2012"xsd:gYear |
| http://purl.uniprot.org/citations/22970133 | http://purl.uniprot.org/core/name | "PLoS One"xsd:string |
| http://purl.uniprot.org/citations/22970133 | http://purl.uniprot.org/core/pages | "e43563"xsd:string |
| http://purl.uniprot.org/citations/22970133 | http://purl.uniprot.org/core/title | "Valosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3."xsd:string |
| http://purl.uniprot.org/citations/22970133 | http://purl.uniprot.org/core/volume | "7"xsd:string |
| http://purl.uniprot.org/citations/22970133 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/22970133 |
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