Matches in SemOpenAlex for { <https://semopenalex.org/work/W3175890269> ?p ?o ?g. }
- W3175890269 endingPage "100914" @default.
- W3175890269 startingPage "100914" @default.
- W3175890269 abstract "GGGGCC (G4C2) hexanucleotide repeat expansions in the endosomal trafficking gene C9orf72 are the most common genetic cause of ALS and frontotemporal dementia. Repeat-associated non-AUG (RAN) translation of this expansion through near-cognate initiation codon usage and internal ribosomal entry generates toxic proteins that accumulate in patients' brains and contribute to disease pathogenesis. The helicase protein DEAH-box helicase 36 (DHX36-G4R1) plays active roles in RNA and DNA G-quadruplex (G4) resolution in cells. As G4C2 repeats are known to form G4 structures in vitro, we sought to determine the impact of manipulating DHX36 expression on repeat transcription and RAN translation. Using a series of luciferase reporter assays both in cells and in vitro, we found that DHX36 depletion suppresses RAN translation in a repeat length-dependent manner, whereas overexpression of DHX36 enhances RAN translation from G4C2 reporter RNAs. Moreover, upregulation of RAN translation that is typically triggered by integrated stress response activation is prevented by loss of DHX36. These results suggest that DHX36 is active in regulating G4C2 repeat translation, providing potential implications for therapeutic development in nucleotide repeat expansion disorders." @default.
- W3175890269 created "2021-07-05" @default.
- W3175890269 creator A5001583322 @default.
- W3175890269 creator A5012674391 @default.
- W3175890269 creator A5028674869 @default.
- W3175890269 creator A5033268821 @default.
- W3175890269 creator A5034812044 @default.
- W3175890269 creator A5044172272 @default.
- W3175890269 creator A5045513566 @default.
- W3175890269 creator A5052049536 @default.
- W3175890269 creator A5055097830 @default.
- W3175890269 creator A5061264765 @default.
- W3175890269 creator A5062235012 @default.
- W3175890269 creator A5068820428 @default.
- W3175890269 creator A5076382619 @default.
- W3175890269 creator A5082894548 @default.
- W3175890269 creator A5089614985 @default.
- W3175890269 date "2021-08-01" @default.
- W3175890269 modified "2023-10-15" @default.
- W3175890269 title "The RNA helicase DHX36–G4R1 modulates C9orf72 GGGGCC hexanucleotide repeat–associated translation" @default.
- W3175890269 cites W10114010 @default.
- W3175890269 cites W1450574606 @default.
- W3175890269 cites W1779265524 @default.
- W3175890269 cites W1887262817 @default.
- W3175890269 cites W1914649288 @default.
- W3175890269 cites W1970949525 @default.
- W3175890269 cites W1975838017 @default.
- W3175890269 cites W1977603207 @default.
- W3175890269 cites W1980083127 @default.
- W3175890269 cites W1983273401 @default.
- W3175890269 cites W1986071629 @default.
- W3175890269 cites W1993656252 @default.
- W3175890269 cites W2006600110 @default.
- W3175890269 cites W2007568107 @default.
- W3175890269 cites W2008410413 @default.
- W3175890269 cites W2008575045 @default.
- W3175890269 cites W2011824034 @default.
- W3175890269 cites W2013488820 @default.
- W3175890269 cites W2015870397 @default.
- W3175890269 cites W2017043601 @default.
- W3175890269 cites W2039247139 @default.
- W3175890269 cites W2041122594 @default.
- W3175890269 cites W2050913592 @default.
- W3175890269 cites W2056022631 @default.
- W3175890269 cites W2056201880 @default.
- W3175890269 cites W2061098625 @default.
- W3175890269 cites W2065219073 @default.
- W3175890269 cites W2079838847 @default.
- W3175890269 cites W2092238715 @default.
- W3175890269 cites W2096719769 @default.
- W3175890269 cites W2114872355 @default.
- W3175890269 cites W2125073277 @default.
- W3175890269 cites W2128133969 @default.
- W3175890269 cites W2137500638 @default.
- W3175890269 cites W2144969895 @default.
- W3175890269 cites W2145347505 @default.
- W3175890269 cites W2150523249 @default.
- W3175890269 cites W2154945114 @default.
- W3175890269 cites W2155773930 @default.
- W3175890269 cites W2166070939 @default.
- W3175890269 cites W2168488590 @default.
- W3175890269 cites W2169745877 @default.
- W3175890269 cites W2190390575 @default.
- W3175890269 cites W2255242978 @default.
- W3175890269 cites W2275411324 @default.
- W3175890269 cites W2289125032 @default.
- W3175890269 cites W2290989326 @default.
- W3175890269 cites W2310237425 @default.
- W3175890269 cites W2319517406 @default.
- W3175890269 cites W2396457105 @default.
- W3175890269 cites W2419112435 @default.
- W3175890269 cites W2427010553 @default.
- W3175890269 cites W2465424457 @default.
- W3175890269 cites W2487331275 @default.
- W3175890269 cites W2502593768 @default.
- W3175890269 cites W2520605819 @default.
- W3175890269 cites W2520749639 @default.
- W3175890269 cites W2531459060 @default.
- W3175890269 cites W2560485036 @default.
- W3175890269 cites W2568319997 @default.
- W3175890269 cites W2569387537 @default.
- W3175890269 cites W2583285404 @default.
- W3175890269 cites W2594983454 @default.
- W3175890269 cites W2611729001 @default.
- W3175890269 cites W2767664549 @default.
- W3175890269 cites W2773167564 @default.
- W3175890269 cites W2777012891 @default.
- W3175890269 cites W2782523843 @default.
- W3175890269 cites W2787234767 @default.
- W3175890269 cites W2803476128 @default.
- W3175890269 cites W2808136147 @default.
- W3175890269 cites W2904358419 @default.
- W3175890269 cites W2908433735 @default.
- W3175890269 cites W2908795173 @default.
- W3175890269 cites W2945274111 @default.
- W3175890269 cites W2948768009 @default.
- W3175890269 cites W2949082553 @default.
- W3175890269 cites W2965188503 @default.