4E59

Crystal structure of GCCGCCGC duplex

4E59 の概要

• エントリーDOI: 10.2210/pdb4e59/pdb
• 関連するPDBエントリー: 4E58
• 分子名称: RNA duplex containing CCG repeats (2 entities in total)
• 機能のキーワード: 3' slippery duplexes, x-linked mental retardation, huntington's disease, myotonic dystrophy type 1, rna, ccg repeats
• 由来する生物種: Homo sapiens
• タンパク質・核酸の鎖数: 2
• 化学式量合計: 5033.14

構造登録者

Kiliszek, A.,Kierzek, R.,Krzyzosiak, W.J.,Rypniewski, W. (登録日: 2012-03-14, 公開日: 2012-12-12, 最終更新日: 2023-09-13)

主引用文献

Kiliszek, A.,Kierzek, R.,Krzyzosiak, W.J.,Rypniewski, W.
Crystallographic characterization of CCG repeats.
Nucleic Acids Res., 40:8155-8162, 2012

PubMed Abstract: CCG repeats are highly over-represented in exons of the human genome. Usually they are located in the 5' UTR but are also abundant in translated sequences. The CCG repeats are associated with three tri-nucleotide repeat disorders: Huntington's disease, myotonic dystrophy type 1 and chromosome X-linked mental retardation (FRAXE). In this study, we present two crystal structures containing double-stranded CCG repeats: one of an RNA in the native form, and one containing LNA nucleotides. Both duplexes form A-helices but with strands slipped in the 5' (native structure) or the 3' direction (LNA-containing structure). As a result, one of two expected C-C pairs is eliminated from the duplex. Each of the three observed C-C pairs interacts differently, forming either one weak H-bond or none. LNA nucleotides have no apparent effect on the helical parameters but the base stacking is increased compared to the native duplex and the distribution of electrostatic potential in the major groove is changed. The CCG crystal structures explain the thermodynamic fragility of CCG runs and throw light on the observation that the MBNL1 protein recognises CCG runs, as well as CUG and CAG, but not the relatively stable CGG repeats.

PubMed: 22718980
DOI: 10.1093/nar/gks557

実験手法

X-RAY DIFFRACTION (1.54 Å)