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http://purl.uniprot.org/citations/30353006http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/30353006http://www.w3.org/2000/01/rdf-schema#comment"TDP-43 is the major disease protein associated with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitinated inclusions (FTLD-TDP). Here we identify the transcriptional elongation factor Ell-a shared component of little elongation complex (LEC) and super elongation complex (SEC)-as a strong modifier of TDP-43-mediated neurodegeneration. Our data indicate select targets of LEC and SEC become upregulated in the fly ALS/FTLD-TDP model. Among them, U12 snRNA and a stress-induced long non-coding RNA Hsrω, functionally contribute to TDP-43-mediated degeneration. We extend the findings of Hsrω, which we identify as a chromosomal target of TDP-43, to show that the human orthologue Sat III is elevated in a human cellular disease model and FTLD-TDP patient tissue. We further demonstrate an interaction between TDP-43 and human ELL2 by co-immunoprecipitation from human cells. These findings reveal important roles of Ell-complexes LEC and SEC in TDP-43-associated toxicity, providing potential therapeutic insight for TDP-43-associated neurodegeneration."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.org/dc/terms/identifier"doi:10.1038/s41467-018-06543-0"xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Kim H.J."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Chung C.Y."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Unger T."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Shilatifard A."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Smith E.R."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Lee V.M."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Bonini N.M."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Kennerdell J.R."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Chen-Plotkin A."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Van Deerlin V."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Berson A."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Porta S."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/author"Sartoris A."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/date"2018"xsd:gYear
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/name"Nat Commun"xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/pages"4406"xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/title"Aberrant activation of non-coding RNA targets of transcriptional elongation complexes contributes to TDP-43 toxicity."xsd:string
http://purl.uniprot.org/citations/30353006http://purl.uniprot.org/core/volume"9"xsd:string
http://purl.uniprot.org/citations/30353006http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/30353006
http://purl.uniprot.org/citations/30353006http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/30353006
http://purl.uniprot.org/uniprot/#_D5SHT8-mappedCitation-30353006http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/30353006
http://purl.uniprot.org/uniprot/#_A0A1W5PXG3-mappedCitation-30353006http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/30353006