AI Article Synopsis
- - Hexanucleotide repeat expansions (G4C2) in the C9orf72 gene are the leading genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) and can form complex secondary structures like G-quadruplexes.
- - These unusual structures at both DNA and RNA levels may trigger processes that lead to the onset of ALS/FTD, making their study important for understanding these diseases.
- - The text also discusses techniques for researching G-quadruplexes and explores potential therapies aimed at targeting them to treat C9-FTD/ALS.
Article Abstract
Hexanucleotide repeat expansions, (G4C2) in the C9orf72 gene are considered as the single most common genetic cause of both frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). (G4C2), either as DNA or the transcribed RNA, can folds into unusual secondary structures, including G-quadruplex, R-loop, I-motif and hairpin. These structural polymorphism at both DNA and RNA levels were proposed to initiate molecular cascade leading to ALS/FTD. G-quadruplexes are composed of stacked G4 tetrads, held by hydrophobic bonds, and is highly stable secondary structure. Here, we covers the structural and functional features of G-quadruplexes with an emphasis on C9orf72-repeat-associated FTD and ALS (C9-FTD/ALS). We also highlighted tools and techniques used to study the G-quadruplexes. Current perspectives for molecules that target G-quadruplexes as potential therapeutic are discussed. Our extensive analysis of structural features of G-quadruplexes will be used for a better understanding of molecular mechanism of C9-FTD/ALS.
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| http://dx.doi.org/10.1016/j.neuint.2016.08.008 | DOI Listing |
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