3NJ6: 0.95 A resolution X-ray structure of (GGCAGCAGCC)2

Citation:
Abstract
CAG repeats occur predominantly in the coding regions of human genes, which suggests their functional importance. In some genes, these sequences can undergo pathogenic expansions leading to neurodegenerative polyglutamine (poly-Q) diseases. The mutant transcripts containing expanded CAG repeats possibly contribute to pathogenesis in addition to the well-known pathogenic effects of mutant proteins. We have analysed two crystal forms of RNA duplexes containing CAG repeats: (GGCAGCAGCC)(2). One of the structures has been determined at atomic resolution (0.95 A) and the other at 1.9 A. The duplexes include non-canonical A-A pairs that fit remarkably well within a regular A-helix. All the adenosines are in the anti-conformation and the only interaction within each A-A pair is a single C2-H2...N1 hydrogen bond. Both adenosines in each A-A pair are shifted towards the major groove, although to different extents; the A which is the H-bond donor stands out more (the 'thumbs-up' conformation). The main effect on the helix conformation is a local unwinding. The CAG repeats and the previously examined CUG structures share a similar pattern of electrostatic charge distribution in the minor groove, which could explain their affinity for the pathogenesis-related MBNL1 protein.
PDB ID: 3NJ6Download
MMDB ID: 84405
PDB Deposition Date: 2010/6/17
Updated in MMDB: 2010/12
Experimental Method:
x-ray diffraction
Resolution: 0.95  Å
Biological Unit for 3NJ6: dimeric; determined by author and by software (PISA)
Molecular Components in 3NJ6
Label Count Molecule
Nucleotide(1 molecule)
2
5'-r(*gp*gp*cp*ap*gp*cp*ap*gp*cp*c)-3'
Molecule annotation
Chemicals (2 molecules)
1
2
* Click molecule labels to explore molecular sequence information.

Citing MMDB
.