1zev

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[[Image:1zev.gif|left|200px]]<br /><applet load="1zev" size="350" color="white" frame="true" align="right" spinBox="true"
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[[Image:1zev.gif|left|200px]]
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caption="1zev, resolution 1.58&Aring;" />
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'''Crystal Structure of a Pathogenic RNA: CUG Repeats'''<br />
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{{Structure
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|PDB= 1zev |SIZE=350|CAPTION= <scene name='initialview01'>1zev</scene>, resolution 1.58&Aring;
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|SITE=
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|LIGAND=
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|ACTIVITY=
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|GENE=
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}}
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'''Crystal Structure of a Pathogenic RNA: CUG Repeats'''
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==Overview==
==Overview==
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==About this Structure==
==About this Structure==
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1ZEV is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/ ]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1ZEV OCA].
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1ZEV is a [[Protein complex]] structure of sequences from [http://en.wikipedia.org/wiki/ ]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1ZEV OCA].
==Reference==
==Reference==
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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:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=16269545 16269545]
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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:[http://www.ncbi.nlm.nih.gov/pubmed/16269545 16269545]
[[Category: Protein complex]]
[[Category: Protein complex]]
[[Category: Berglund, J A.]]
[[Category: Berglund, J A.]]
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[[Category: Mooers, B H.]]
[[Category: Mooers, B H.]]
[[Category: causitive agent of myotonic dystrophy type i]]
[[Category: causitive agent of myotonic dystrophy type i]]
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[[Category: triplet repeats]]
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[[Category: triplet repeat]]
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[[Category: uu mismatches]]
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[[Category: uu mismatch]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 16:14:50 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 15:34:35 2008''

Revision as of 13:34, 20 March 2008


PDB ID 1zev

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, resolution 1.58Å
Coordinates: save as pdb, mmCIF, xml



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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