1zev
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(New page: 200px<br /><applet load="1zev" size="450" color="white" frame="true" align="right" spinBox="true" caption="1zev, resolution 1.58Å" /> '''Crystal Structure of...)
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Revision as of 02:43, 25 November 2007
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Crystal Structure of a Pathogenic RNA: CUG Repeats
Overview
Myotonic dystrophy (DM) type 1 is associated with an expansion of (>50), CTG repeats within the 3' untranslated region (UTR) of the dystrophin, myotonin protein kinase gene (dmpk). In the corresponding mRNA transcript, the CUG repeats form an extended stem-loop structure. The double-stranded, RNA of the stem sequesters RNA binding proteins away from their normal, cellular targets resulting in aberrant transcription, alternative splicing, patterns, or both, thereby leading to DM. To better understand the, structural basis of DM type 1, we determined to 1.58-A resolution the, x-ray crystal structure of an 18-bp RNA containing six CUG repeats. The, CUG repeats form antiparallel double-stranded helices that stack, end-on-end in the crystal to form infinite, pseudocontinuous helices, similar to the long CUG stem loops formed by the expanded CUG repeats in, DM type 1. The CUG helix is very similar in structure to A-form RNA with, the exception of the unique U-U mismatches. This structure provides a, high-resolution view of a toxic, trinucleotide repeat RNA.
About this Structure
1ZEV is a Protein complex structure of sequences from [1]. Full crystallographic information is available from OCA.
Reference
The structural basis of myotonic dystrophy from the crystal structure of CUG repeats., Mooers BH, Logue JS, Berglund JA, Proc Natl Acad Sci U S A. 2005 Nov 15;102(46):16626-31. Epub 2005 Nov 3. PMID:16269545
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