| http://purl.uniprot.org/citations/27146268 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/27146268 | http://www.w3.org/2000/01/rdf-schema#comment | "The disruption of protein quality control networks is central to pathology in Huntington's disease (HD) and other neurodegenerative disorders. The aberrant accumulation of insoluble high-molecular-weight protein complexes containing the Huntingtin (HTT) protein and SUMOylated protein corresponds to disease manifestation. We previously identified an HTT-selective E3 SUMO ligase, PIAS1, that regulates HTT accumulation and SUMO modification in cells. Here we investigated whether PIAS1 modulation in neurons alters HD-associated phenotypes in vivo. Instrastriatal injection of a PIAS1-directed miRNA significantly improved behavioral phenotypes in rapidly progressing mutant HTT (mHTT) fragment R6/2 mice. PIAS1 reduction prevented the accumulation of mHTT and SUMO- and ubiquitin-modified proteins, increased synaptophysin levels, and normalized key inflammatory markers. In contrast, PIAS1 overexpression exacerbated mHTT-associated phenotypes and aberrant protein accumulation. These results confirm the association between aberrant accumulation of expanded polyglutamine-dependent insoluble protein species and pathogenesis, and they link phenotypic benefit to reduction of these species through PIAS1 modulation."xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.neuron.2016.03.016"xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/author | "Davidson B.L."xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/author | "Steffan J.S."xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/author | "Thompson L.M."xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/author | "O'Rourke J.G."xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/author | "Reidling J.C."xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/author | "Monteys A.M."xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/author | "Ochaba J."xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/date | "2016"xsd:gYear |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/name | "Neuron"xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/pages | "507-520"xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/title | "PIAS1 Regulates Mutant Huntingtin Accumulation and Huntington's Disease-Associated Phenotypes In Vivo."xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://purl.uniprot.org/core/volume | "90"xsd:string |
| http://purl.uniprot.org/citations/27146268 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/27146268 |
| http://purl.uniprot.org/citations/27146268 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/27146268 |
| http://purl.uniprot.org/uniprot/#_A0A1L1SQX9-mappedCitation-27146268 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/27146268 |
| http://purl.uniprot.org/uniprot/#_A0A1L1SRS2-mappedCitation-27146268 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/27146268 |
| http://purl.uniprot.org/uniprot/#_A4ZVS7-mappedCitation-27146268 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/27146268 |
| http://purl.uniprot.org/uniprot/#_A4ZVS8-mappedCitation-27146268 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/27146268 |
| http://purl.uniprot.org/uniprot/#_O75925-mappedCitation-27146268 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/27146268 |
| http://purl.uniprot.org/uniprot/#_Q3U556-mappedCitation-27146268 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/27146268 |
| http://purl.uniprot.org/uniprot/#_Q2M4G9-mappedCitation-27146268 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/27146268 |
| http://purl.uniprot.org/uniprot/#_O88907-mappedCitation-27146268 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/27146268 |