1zev

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{{STRUCTURE_1zev| PDB=1zev | SCENE= }}
{{STRUCTURE_1zev| PDB=1zev | SCENE= }}
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'''Crystal Structure of a Pathogenic RNA: CUG Repeats'''
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===Crystal Structure of a Pathogenic RNA: CUG Repeats===
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==Overview==
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Myotonic dystrophy (DM) type 1 is associated with an expansion of (&gt;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.
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(as it appears on PubMed at http://www.pubmed.gov), where 16269545 is the PubMed ID number.
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{{ABSTRACT_PUBMED_16269545}}
==About this Structure==
==About this Structure==
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[[Category: Triplet repeat]]
[[Category: Triplet repeat]]
[[Category: Uu mismatch]]
[[Category: Uu mismatch]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 17:32:15 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Jul 10 12:38:55 2008''

Revision as of 09:38, 10 July 2008

Image:1zev.png

Template:STRUCTURE 1zev

Crystal Structure of a Pathogenic RNA: CUG Repeats

Template:ABSTRACT PUBMED 16269545

About this Structure

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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