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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caption="1zev, resolution 1.58&Aring;" />
 
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'''Crystal Structure of a Pathogenic RNA: CUG Repeats'''<br />
 
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==Overview==
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==Crystal Structure of a Pathogenic RNA: CUG Repeats==
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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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<StructureSection load='1zev' size='340' side='right'caption='[[1zev]], [[Resolution|resolution]] 1.58&Aring;' scene=''>
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== Structural highlights ==
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==About this Structure==
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<table><tr><td colspan='2'>[[1zev]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1ZEV OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1ZEV FirstGlance]. <br>
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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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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.58&#8491;</td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1zev FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1zev OCA], [https://pdbe.org/1zev PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1zev RCSB], [https://www.ebi.ac.uk/pdbsum/1zev PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1zev ProSAT]</span></td></tr>
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==Reference==
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</table>
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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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__TOC__
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[[Category: Protein complex]]
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</StructureSection>
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[[Category: Berglund, J A.]]
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[[Category: Large Structures]]
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[[Category: Logue, J S.]]
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[[Category: Berglund JA]]
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[[Category: Mooers, B H.]]
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[[Category: Logue JS]]
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[[Category: causitive agent of myotonic dystrophy type i]]
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[[Category: Mooers BH]]
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[[Category: triplet repeats]]
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[[Category: uu mismatches]]
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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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Current revision

Crystal Structure of a Pathogenic RNA: CUG Repeats

PDB ID 1zev

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