http://purl.uniprot.org/citations/32653676 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/32653676 | http://www.w3.org/2000/01/rdf-schema#comment | "Fragile X associated tremor/ataxia syndrome (FXTAS) is a late-onset neurodegenerative disorder caused by expansion of CGG repeats in the 5' UTR of the fragile X mental retardation 1 (FMR1) gene. Using the well-established FXTAS Drosophila model, we performed a high-throughput chemical screen using 3200 small molecules. NSC363998 was identified to suppress the neurodegeneration caused by riboCGG (rCGG) repeats. Three predicted targets of a NSC363998 derivative are isopeptidases in the neddylation pathway and could modulate the neurotoxicity caused by the rCGG repeats. Decreasing levels of neddylation resulted in enhancing neurodegeneration phenotypes, while up-regulation could rescue the phenotypes. Furthermore, known neddylation substrates, Cul3 and Vhl, and their downstream target, Sima, were found to modulate rCGG90-dependent neurotoxicity. Our results suggest that altered neddylation activity can modulate the rCGG repeat-mediated toxicity by regulating Sima protein levels, which could serve as a potential therapeutic target for FXTAS."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.nbd.2020.105013"xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Chen J."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Duan R."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "He H."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Li J."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Luo S."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Lin Y."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Yu L."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Zhao J."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Jin P."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Deng J."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Xue J."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Chen J.'"xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/author | "Allen E.G."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/date | "2020"xsd:gYear |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/name | "Neurobiol Dis"xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/pages | "105013"xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/title | "Neddylation activity modulates the neurodegeneration associated with fragile X associated tremor/ataxia syndrome (FXTAS) through regulating Sima."xsd:string |
http://purl.uniprot.org/citations/32653676 | http://purl.uniprot.org/core/volume | "143"xsd:string |
http://purl.uniprot.org/citations/32653676 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/32653676 |
http://purl.uniprot.org/citations/32653676 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/32653676 |
http://purl.uniprot.org/uniprot/#_A0A0B4KHI3-mappedCitation-32653676 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/32653676 |
http://purl.uniprot.org/uniprot/#_A0A0B4KHM2-mappedCitation-32653676 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/32653676 |