FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2040271470> ?p ?o ?g. }

• W2040271470 endingPage "e752" @default.
• W2040271470 startingPage "e752" @default.
• W2040271470 abstract "Several inherited neurodegenerative disorders are caused by CAG trinucleotide repeat expansions, which can be located either in the coding region or in the untranslated region (UTR) of the respective genes. Polyglutamine diseases (polyQ diseases) are caused by an expansion of a stretch of CAG repeats within the coding region, translating into a polyQ tract. The polyQ tract expansions result in conformational changes, eventually leading to aggregate formation. It is widely believed that the aggregation of polyQ proteins is linked with disease development. In addition, in the last couple of years, it has been shown that RNA-mediated mechanisms also have a profound role in neurotoxicity in both polyQ diseases and diseases caused by elongated CAG repeat motifs in their UTRs. Here, we review the different molecular mechanisms assigned to mRNAs with expanded CAG repeats. One aspect is the mRNA folding of CAG repeats. Furthermore, pathogenic mechanisms assigned to CAG repeat mRNAs are discussed. First, we discuss mechanisms that involve the sequestration of the diverse proteins to the expanded CAG repeat mRNA molecules. As a result of this, several cellular mechanisms are aberrantly regulated. These include the sequestration of MBNL1, leading to misregulated splicing; sequestration of nucleolin, leading to reduced cellular rRNA; and sequestration of proteins of the siRNA machinery, resulting in the production of short silencing RNAs that affect gene expression. Second, we discuss the effect of expanded CAG repeats on the subcellular localization, transcription and translation of the CAG repeat mRNA itself. Here we focus on the MID1 protein complex that triggers an increased translation of expanded CAG repeat mRNAs and a mechanism called repeat-associated non-ATG translation, which leads to proteins aberrantly translated from CAG repeat mRNAs. In addition, therapeutic approaches for CAG repeat disorders are discussed. Together, all the findings summarized here show that mutant mRNA has a fundamental role in the pathogenesis of CAG repeat diseases." @default.
• W2040271470 created "2016-06-24" @default.
• W2040271470 creator A5022926011 @default.
• W2040271470 creator A5030833447 @default.
• W2040271470 creator A5031422181 @default.
• W2040271470 creator A5036026487 @default.
• W2040271470 creator A5058004814 @default.
• W2040271470 date "2013-08-01" @default.
• W2040271470 modified "2023-10-07" @default.
• W2040271470 title "Mechanisms of RNA-induced toxicity in CAG repeat disorders" @default.
• W2040271470 cites W1513749224 @default.
• W2040271470 cites W1576464279 @default.
• W2040271470 cites W1585249222 @default.
• W2040271470 cites W1647075334 @default.
• W2040271470 cites W1686333409 @default.
• W2040271470 cites W1916491745 @default.
• W2040271470 cites W1965568102 @default.
• W2040271470 cites W1971396436 @default.
• W2040271470 cites W1972088609 @default.
• W2040271470 cites W1976420135 @default.
• W2040271470 cites W1978262065 @default.
• W2040271470 cites W1978352073 @default.
• W2040271470 cites W1979190471 @default.
• W2040271470 cites W1979662111 @default.
• W2040271470 cites W1980521034 @default.
• W2040271470 cites W1980624807 @default.
• W2040271470 cites W1981812360 @default.
• W2040271470 cites W1986022917 @default.
• W2040271470 cites W1992307025 @default.
• W2040271470 cites W1995397443 @default.
• W2040271470 cites W1999617545 @default.
• W2040271470 cites W2000511338 @default.
• W2040271470 cites W2002009217 @default.
• W2040271470 cites W2004219186 @default.
• W2040271470 cites W2005446612 @default.
• W2040271470 cites W2005539608 @default.
• W2040271470 cites W2012145036 @default.
• W2040271470 cites W2013963839 @default.
• W2040271470 cites W2014520750 @default.
• W2040271470 cites W2015419750 @default.
• W2040271470 cites W2016281944 @default.
• W2040271470 cites W2017255898 @default.
• W2040271470 cites W2029984090 @default.
• W2040271470 cites W2036122182 @default.
• W2040271470 cites W2037064308 @default.
• W2040271470 cites W2037288833 @default.
• W2040271470 cites W2042427251 @default.
• W2040271470 cites W2047391267 @default.
• W2040271470 cites W2050476079 @default.
• W2040271470 cites W2051489116 @default.
• W2040271470 cites W2051575011 @default.
• W2040271470 cites W2052857239 @default.
• W2040271470 cites W2057739264 @default.
• W2040271470 cites W2058448931 @default.
• W2040271470 cites W2063685191 @default.
• W2040271470 cites W2064791021 @default.
• W2040271470 cites W2066863701 @default.
• W2040271470 cites W2067317023 @default.
• W2040271470 cites W2070224195 @default.
• W2040271470 cites W2074695844 @default.
• W2040271470 cites W2076791245 @default.
• W2040271470 cites W2076959245 @default.
• W2040271470 cites W2081280332 @default.
• W2040271470 cites W2081635886 @default.
• W2040271470 cites W2082623267 @default.
• W2040271470 cites W2082759452 @default.
• W2040271470 cites W2083076780 @default.
• W2040271470 cites W2083824522 @default.
• W2040271470 cites W2085345635 @default.
• W2040271470 cites W2087063545 @default.
• W2040271470 cites W2087166511 @default.
• W2040271470 cites W2092831145 @default.
• W2040271470 cites W2092938083 @default.
• W2040271470 cites W2093982999 @default.
• W2040271470 cites W2095685874 @default.
• W2040271470 cites W2098723544 @default.
• W2040271470 cites W2098934468 @default.
• W2040271470 cites W2099673459 @default.
• W2040271470 cites W2100275934 @default.
• W2040271470 cites W2103116226 @default.
• W2040271470 cites W2106012958 @default.
• W2040271470 cites W2106490406 @default.
• W2040271470 cites W2107007950 @default.
• W2040271470 cites W2108092527 @default.
• W2040271470 cites W2108570063 @default.
• W2040271470 cites W2113918626 @default.
• W2040271470 cites W2114410594 @default.
• W2040271470 cites W2117724716 @default.
• W2040271470 cites W2117991391 @default.
• W2040271470 cites W2118428181 @default.
• W2040271470 cites W2121104406 @default.
• W2040271470 cites W2121726191 @default.
• W2040271470 cites W2123193865 @default.
• W2040271470 cites W2127313039 @default.
• W2040271470 cites W2128234733 @default.
• W2040271470 cites W2128882718 @default.
• W2040271470 cites W2131173813 @default.
• W2040271470 cites W2131228505 @default.

• next