| http://purl.uniprot.org/citations/30038412 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/30038412 | http://www.w3.org/2000/01/rdf-schema#comment | "Induced pluripotent stem cells (iPSCs) undergo unlimited self-renewal while maintaining their potential to differentiate into post-mitotic cells with an intact proteome. As such, iPSCs suppress the aggregation of polyQ-expanded huntingtin (HTT), the mutant protein underlying Huntington's disease (HD). Here we show that proteasome activity determines HTT levels, preventing polyQ-expanded aggregation in iPSCs from HD patients (HD-iPSCs). iPSCs exhibit high levels of UBR5, a ubiquitin ligase required for proteasomal degradation of both normal and mutant HTT. Conversely, loss of UBR5 increases HTT levels and triggers polyQ-expanded aggregation in HD-iPSCs. Moreover, UBR5 knockdown hastens polyQ-expanded aggregation and neurotoxicity in invertebrate models. Notably, UBR5 overexpression induces polyubiquitination and degradation of mutant HTT, reducing polyQ-expanded aggregates in HD-cell models. Besides HTT levels, intrinsic enhanced UBR5 expression determines global proteostasis of iPSCs preventing the aggregation of misfolded proteins ensued from normal metabolism. Thus, our findings indicate UBR5 as a modulator of super-vigilant proteostasis of iPSCs."xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.org/dc/terms/identifier | "doi:10.1038/s41467-018-05320-3"xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/author | "Lee H.J."xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/author | "Hoppe T."xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/author | "Pokrzywa W."xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/author | "Koyuncu S."xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/author | "Vilchez D."xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/author | "Fatima A."xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/author | "Saez I."xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/author | "Gutierrez-Garcia R."xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/date | "2018"xsd:gYear |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/name | "Nat Commun"xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/pages | "2886"xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/title | "The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington's disease patients."xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://purl.uniprot.org/core/volume | "9"xsd:string |
| http://purl.uniprot.org/citations/30038412 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/30038412 |
| http://purl.uniprot.org/citations/30038412 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/30038412 |
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