| http://purl.uniprot.org/citations/23804749 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/23804749 | http://www.w3.org/2000/01/rdf-schema#comment | "Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive and selective loss of motor neurons. The discovery of mutations in the gene encoding an RNA-binding protein, TAR DNA-binding protein of 43 kD (TDP-43), in familial ALS, strongly implicated abnormalities in RNA processing in the pathogenesis of ALS, although the mechanisms whereby TDP-43 leads to neurodegeneration remain elusive. To clarify the mechanism of degeneration caused by TDP-43, we generated transgenic Drosophila melanogaster expressing a series of systematically modified human TDP-43 genes in the retinal photoreceptor neurons. Overexpression of wild-type TDP-43 resulted in vacuolar degeneration of the photoreceptor neurons associated with thinning of the retina, which was significantly exacerbated by mutations of TDP-43 linked to familial ALS or disrupting its nuclear localization signal (NLS). Remarkably, these degenerative phenotypes were completely normalized by addition of a mutation or deletion of the RNA recognition motif that abolishes the RNA binding ability of TDP-43. Altogether, our results suggest that RNA binding is key to the neurodegeneration caused by overexpression of TDP-43, and that abnormalities in RNA processing may be crucial to the pathogenesis of TDP-43 proteinopathy."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.org/dc/terms/identifier | "doi:10.1093/hmg/ddt296"xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/author | "Hashimoto T."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/author | "Nagata Y."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/author | "Miura M."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/author | "Iwatsubo T."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/author | "Tsuji S."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/author | "Wakabayashi T."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/author | "Chihara T."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/author | "Kuranaga E."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/author | "Kunugi H."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/author | "Ihara R."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/author | "Matsukawa K."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/date | "2013"xsd:gYear |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/name | "Hum Mol Genet"xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/pages | "4474-4484"xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/title | "RNA binding mediates neurotoxicity in the transgenic Drosophila model of TDP-43 proteinopathy."xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://purl.uniprot.org/core/volume | "22"xsd:string |
| http://purl.uniprot.org/citations/23804749 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/23804749 |
| http://purl.uniprot.org/citations/23804749 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/23804749 |
| http://purl.uniprot.org/uniprot/#_A0A0A0MSV7-mappedCitation-23804749 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/23804749 |
| http://purl.uniprot.org/uniprot/#_A0A0A0N0L3-mappedCitation-23804749 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/23804749 |
| http://purl.uniprot.org/uniprot/#_A0A0A0N0L6-mappedCitation-23804749 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/23804749 |
| http://purl.uniprot.org/uniprot/#_A0A0A0N0M3-mappedCitation-23804749 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/23804749 |