| http://purl.uniprot.org/citations/26130692 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/26130692 | http://www.w3.org/2000/01/rdf-schema#comment | "TAR DNA-binding protein 43 (TDP-43) is a major disease protein in amyotrophic lateral sclerosis (ALS) and related neurodegenerative diseases. Both the cytoplasmic accumulation of toxic ubiquitinated and hyperphosphorylated TDP-43 fragments and the loss of normal TDP-43 from the nucleus may contribute to the disease progression by impairing normal RNA and protein homeostasis. Therefore, both the removal of pathological protein and the rescue of TDP-43 mislocalization may be critical for halting or reversing TDP-43 proteinopathies. Here, we report poly(A)-binding protein nuclear 1 (PABPN1) as a novel TDP-43 interaction partner that acts as a potent suppressor of TDP-43 toxicity. Overexpression of full-length PABPN1 but not a truncated version lacking the nuclear localization signal protects from pathogenic TDP-43-mediated toxicity, promotes the degradation of pathological TDP-43 and restores normal solubility and nuclear localization of endogenous TDP-43. Reduced levels of PABPN1 enhances the phenotypes in several cell culture and Drosophila models of ALS and results in the cytoplasmic mislocalization of TDP-43. Moreover, PABPN1 rescues the dysregulated stress granule (SG) dynamics and facilitates the removal of persistent SGs in TDP-43-mediated disease conditions. These findings demonstrate a role for PABPN1 in rescuing several cytopathological features of TDP-43 proteinopathy by increasing the turnover of pathologic proteins."xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.org/dc/terms/identifier | "doi:10.1093/hmg/ddv238"xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/author | "Chou C.C."xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/author | "Zhang Y."xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/author | "Yamada S."xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/author | "Williams K.R."xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/author | "Zarnescu D.C."xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/author | "Mo B."xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/author | "Rossoll W."xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/author | "Alexeeva O.M."xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/author | "Pribadi A."xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/date | "2015"xsd:gYear |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/name | "Hum Mol Genet"xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/pages | "5154-5173"xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/title | "PABPN1 suppresses TDP-43 toxicity in ALS disease models."xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://purl.uniprot.org/core/volume | "24"xsd:string |
| http://purl.uniprot.org/citations/26130692 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/26130692 |
| http://purl.uniprot.org/citations/26130692 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/26130692 |
| http://purl.uniprot.org/uniprot/#_A0A087WQ63-mappedCitation-26130692 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26130692 |
| http://purl.uniprot.org/uniprot/#_A0A087WQA5-mappedCitation-26130692 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26130692 |
| http://purl.uniprot.org/uniprot/#_A0A0A0MSV7-mappedCitation-26130692 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26130692 |
| http://purl.uniprot.org/uniprot/#_A0A0A0N0L3-mappedCitation-26130692 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26130692 |
| http://purl.uniprot.org/uniprot/#_A0A087WNY6-mappedCitation-26130692 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26130692 |
| http://purl.uniprot.org/uniprot/#_A0A087WP57-mappedCitation-26130692 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26130692 |