1zev
From Proteopedia
(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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| - | [[Image:1zev.gif|left|200px]]<br /><applet load="1zev" size=" | + | [[Image:1zev.gif|left|200px]]<br /><applet load="1zev" size="350" color="white" frame="true" align="right" spinBox="true" |
caption="1zev, resolution 1.58Å" /> | caption="1zev, resolution 1.58Å" /> | ||
'''Crystal Structure of a Pathogenic RNA: CUG Repeats'''<br /> | '''Crystal Structure of a Pathogenic RNA: CUG Repeats'''<br /> | ||
==Overview== | ==Overview== | ||
| - | Myotonic dystrophy (DM) type 1 is associated with an expansion of (>50) | + | 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== | ==About this Structure== | ||
| - | 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:// | + | 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]. |
==Reference== | ==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:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=16269545 16269545] | 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] | ||
[[Category: Protein complex]] | [[Category: Protein complex]] | ||
| - | [[Category: Berglund, J | + | [[Category: Berglund, J A.]] |
| - | [[Category: Logue, J | + | [[Category: Logue, J S.]] |
| - | [[Category: Mooers, B | + | [[Category: Mooers, B H.]] |
[[Category: causitive agent of myotonic dystrophy type i]] | [[Category: causitive agent of myotonic dystrophy type i]] | ||
[[Category: triplet repeats]] | [[Category: triplet repeats]] | ||
[[Category: uu mismatches]] | [[Category: uu mismatches]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 16:14:50 2008'' |
Revision as of 14:14, 21 February 2008
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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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