Article ; Online: Metformin inhibits RAN translation through PKR pathway and mitigates disease in
Proceedings of the National Academy of Sciences of the United States of America
2020 Volume 117, Issue 31, Page(s) 18591–18599
Abstract: 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). ... ...
| Abstract | 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 G |
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| MeSH term(s) | Amyotrophic Lateral Sclerosis/metabolism ; Animals ; Brain/metabolism ; Brain/pathology ; C9orf72 Protein/genetics ; C9orf72 Protein/metabolism ; Disease Models, Animal ; Frontotemporal Dementia/metabolism ; Humans ; Metformin/pharmacology ; Mice ; Mice, Transgenic ; Microsatellite Repeats/genetics ; Protein Biosynthesis/drug effects ; eIF-2 Kinase/genetics ; eIF-2 Kinase/metabolism | |||||
| Chemical Substances | C9orf72 Protein ; Metformin (9100L32L2N) ; eIF-2 Kinase (EC 2.7.11.1) | |||||
| Language | English | |||||
| Publishing date | 2020-07-20 | |||||
| Publishing country | United States | |||||
| Document type | Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, Non-P.H.S. | |||||
| ZDB-ID | 209104-5 | |||||
| ISSN | 1091-6490 ; 0027-8424 | |||||
| ISSN (online) | 1091-6490 | |||||
| ISSN | 0027-8424 | |||||
| DOI | 10.1073/pnas.2005748117 | |||||
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| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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