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• W4200339992 abstract "Noncoding RNAs play regulatory roles in physiopathology, but their involvement in neurodevelopmental diseases is poorly understood. Rett syndrome is a severe, progressive neurodevelopmental disorder linked to loss-of-function mutations of the MeCP2 gene for which no cure is yet available. Analysis of the noncoding RNA profile corresponding to the brain-abundant circular RNA (circRNA) and transcribed-ultraconserved region (T-UCR) populations in a mouse model of the disease reveals widespread dysregulation and enrichment in glutamatergic excitatory signaling and microtubule cytoskeleton pathways of the corresponding host genes. Proteomic analysis of hippocampal samples from affected individuals confirms abnormal levels of several cytoskeleton-related proteins together with key alterations in neurotransmission. Importantly, the glutamate receptor GRIA3 gene displays altered biogenesis in affected individuals and in vitro human cells and is influenced by expression of two ultraconserved RNAs. We also describe post-transcriptional regulation of SIRT2 by circRNAs, which modulates acetylation and total protein levels of GluR-1. As a consequence, both regulatory mechanisms converge on the biogenesis of AMPA receptors, with an effect on neuronal differentiation. In both cases, the noncoding RNAs antagonize MeCP2-directed regulation. Our findings indicate that noncoding transcripts may contribute to key alterations in Rett syndrome and are not only useful tools for revealing dysregulated processes but also molecules of biomarker value. Noncoding RNAs play regulatory roles in physiopathology, but their involvement in neurodevelopmental diseases is poorly understood. Rett syndrome is a severe, progressive neurodevelopmental disorder linked to loss-of-function mutations of the MeCP2 gene for which no cure is yet available. Analysis of the noncoding RNA profile corresponding to the brain-abundant circular RNA (circRNA) and transcribed-ultraconserved region (T-UCR) populations in a mouse model of the disease reveals widespread dysregulation and enrichment in glutamatergic excitatory signaling and microtubule cytoskeleton pathways of the corresponding host genes. Proteomic analysis of hippocampal samples from affected individuals confirms abnormal levels of several cytoskeleton-related proteins together with key alterations in neurotransmission. Importantly, the glutamate receptor GRIA3 gene displays altered biogenesis in affected individuals and in vitro human cells and is influenced by expression of two ultraconserved RNAs. We also describe post-transcriptional regulation of SIRT2 by circRNAs, which modulates acetylation and total protein levels of GluR-1. As a consequence, both regulatory mechanisms converge on the biogenesis of AMPA receptors, with an effect on neuronal differentiation. In both cases, the noncoding RNAs antagonize MeCP2-directed regulation. Our findings indicate that noncoding transcripts may contribute to key alterations in Rett syndrome and are not only useful tools for revealing dysregulated processes but also molecules of biomarker value." @default.
• W4200339992 created "2021-12-31" @default.
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• W4200339992 date "2022-03-01" @default.
• W4200339992 modified "2023-10-18" @default.
• W4200339992 title "Analysis of the circRNA and T-UCR populations identifies convergent pathways in mouse and human models of Rett syndrome" @default.
• W4200339992 cites W1023820744 @default.
• W4200339992 cites W1541974580 @default.
• W4200339992 cites W1559742681 @default.
• W4200339992 cites W1566289870 @default.
• W4200339992 cites W1587655960 @default.
• W4200339992 cites W1605270587 @default.
• W4200339992 cites W1827431288 @default.
• W4200339992 cites W1964917360 @default.
• W4200339992 cites W1971735392 @default.
• W4200339992 cites W1974814280 @default.
• W4200339992 cites W1975791071 @default.
• W4200339992 cites W1977709885 @default.
• W4200339992 cites W1979332361 @default.
• W4200339992 cites W1981640964 @default.
• W4200339992 cites W1984124345 @default.
• W4200339992 cites W1985567838 @default.
• W4200339992 cites W1991082489 @default.
• W4200339992 cites W1996457750 @default.
• W4200339992 cites W1997517510 @default.
• W4200339992 cites W2000670569 @default.
• W4200339992 cites W2001657520 @default.
• W4200339992 cites W2003463874 @default.
• W4200339992 cites W2006363083 @default.
• W4200339992 cites W2013405241 @default.
• W4200339992 cites W2015748323 @default.
• W4200339992 cites W2018175199 @default.
• W4200339992 cites W2022959246 @default.
• W4200339992 cites W2025330173 @default.
• W4200339992 cites W2031452891 @default.
• W4200339992 cites W2054891772 @default.
• W4200339992 cites W2055353291 @default.
• W4200339992 cites W2055667548 @default.
• W4200339992 cites W2056985722 @default.
• W4200339992 cites W2058835062 @default.
• W4200339992 cites W2061123111 @default.
• W4200339992 cites W2063840478 @default.
• W4200339992 cites W2063987809 @default.
• W4200339992 cites W2067410483 @default.
• W4200339992 cites W2069075798 @default.
• W4200339992 cites W2069495624 @default.
• W4200339992 cites W2070050178 @default.
• W4200339992 cites W2076304606 @default.
• W4200339992 cites W2077270545 @default.
• W4200339992 cites W2083421199 @default.
• W4200339992 cites W2083887481 @default.
• W4200339992 cites W2088327864 @default.
• W4200339992 cites W2088939471 @default.
• W4200339992 cites W2092133261 @default.
• W4200339992 cites W2094867378 @default.
• W4200339992 cites W2095799789 @default.
• W4200339992 cites W2100990361 @default.
• W4200339992 cites W2101182085 @default.
• W4200339992 cites W2105916826 @default.
• W4200339992 cites W2121145891 @default.
• W4200339992 cites W2122928709 @default.
• W4200339992 cites W2124413347 @default.
• W4200339992 cites W2126823476 @default.
• W4200339992 cites W2126938291 @default.
• W4200339992 cites W2127311839 @default.
• W4200339992 cites W2133130307 @default.
• W4200339992 cites W2141831115 @default.
• W4200339992 cites W2146512944 @default.
• W4200339992 cites W2166251028 @default.
• W4200339992 cites W2168500744 @default.
• W4200339992 cites W2169760625 @default.
• W4200339992 cites W2178051376 @default.
• W4200339992 cites W2179188078 @default.
• W4200339992 cites W2184997675 @default.
• W4200339992 cites W2263028779 @default.
• W4200339992 cites W2326363926 @default.
• W4200339992 cites W2345356016 @default.
• W4200339992 cites W2353377670 @default.
• W4200339992 cites W2403382854 @default.
• W4200339992 cites W2463052690 @default.
• W4200339992 cites W2465981615 @default.
• W4200339992 cites W2469126403 @default.

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