| http://purl.uniprot.org/citations/32690681 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/32690681 | http://www.w3.org/2000/01/rdf-schema#comment | "Repeat associated non-AUG (RAN) translation is found in a growing number of microsatellite expansion diseases, but the mechanisms remain unclear. We show that RAN translation is highly regulated by the double-stranded RNA-dependent protein kinase (PKR). In cells, structured CAG, CCUG, CAGG, and G4C2 expansion RNAs activate PKR, which leads to increased levels of multiple RAN proteins. Blocking PKR using PKR-K296R, the TAR RNA binding protein or PKR-KO cells, reduces RAN protein levels. p-PKR is elevated in C9orf72 ALS/FTD human and mouse brains, and inhibiting PKR in C9orf72 BAC transgenic mice using AAV-PKR-K296R or the Food and Drug Administration (FDA)-approved drug metformin, decreases RAN proteins, and improves behavior and pathology. In summary, targeting PKR, including by use of metformin, is a promising therapeutic approach for C9orf72 ALS/FTD and other expansion diseases."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.org/dc/terms/identifier | "doi:10.1073/pnas.2005748117"xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Li J."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Guo S."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Nguyen L."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Sonenberg N."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Zu T."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Ranum L.P.W."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Engelbrecht A."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Golde T.E."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Chakrabarty P."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Ryskamp D.A."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Bardhi O."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Khoramian Tusi S."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/author | "Klippel K."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/date | "2020"xsd:gYear |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/name | "Proc Natl Acad Sci U S A"xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/pages | "18591-18599"xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/title | "Metformin inhibits RAN translation through PKR pathway and mitigates disease in C9orf72 ALS/FTD mice."xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://purl.uniprot.org/core/volume | "117"xsd:string |
| http://purl.uniprot.org/citations/32690681 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/32690681 |
| http://purl.uniprot.org/citations/32690681 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/32690681 |
| http://purl.uniprot.org/uniprot/#_A2ANZ2-mappedCitation-32690681 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/32690681 |
| http://purl.uniprot.org/uniprot/#_Q8K3B2-mappedCitation-32690681 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/32690681 |