1eg4
From Proteopedia
(New page: 200px<br /> <applet load="1eg4" size="450" color="white" frame="true" align="right" spinBox="true" caption="1eg4, resolution 2.0Å" /> '''STRUCTURE OF A DYSTR...) |
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| - | [[Image:1eg4. | + | [[Image:1eg4.jpg|left|200px]]<br /><applet load="1eg4" size="350" color="white" frame="true" align="right" spinBox="true" |
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caption="1eg4, resolution 2.0Å" /> | caption="1eg4, resolution 2.0Å" /> | ||
'''STRUCTURE OF A DYSTROPHIN WW DOMAIN FRAGMENT IN COMPLEX WITH A BETA-DYSTROGLYCAN PEPTIDE'''<br /> | '''STRUCTURE OF A DYSTROPHIN WW DOMAIN FRAGMENT IN COMPLEX WITH A BETA-DYSTROGLYCAN PEPTIDE'''<br /> | ||
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==About this Structure== | ==About this Structure== | ||
| - | 1EG4 is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http:// | + | 1EG4 is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1EG4 OCA]. |
==Reference== | ==Reference== | ||
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[[Category: ww domain]] | [[Category: ww domain]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri Feb 15 15:43:09 2008'' |
Revision as of 13:43, 15 February 2008
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STRUCTURE OF A DYSTROPHIN WW DOMAIN FRAGMENT IN COMPLEX WITH A BETA-DYSTROGLYCAN PEPTIDE
Contents |
Overview
Dystrophin and beta-dystroglycan are components of the, dystrophin-glycoprotein complex (DGC), a multimolecular assembly that, spans the cell membrane and links the actin cytoskeleton to the, extracellular basal lamina. Defects in the dystrophin gene are the cause, of Duchenne and Becker muscular dystrophies. The C-terminal region of, dystrophin binds the cytoplasmic tail of beta-dystroglycan, in part, through the interaction of its WW domain with a proline-rich motif in the, tail of beta-dystroglycan. Here we report the crystal structure of this, portion of dystrophin in complex with the proline-rich binding site in, beta-dystroglycan. The structure shows that the dystrophin WW domain is, embedded in an adjacent helical region that contains two EF-hand-like, domains. The beta-dystroglycan peptide binds a composite surface formed by, the WW domain and one of these EF-hands. Additionally, the structure, reveals striking similarities in the mechanisms of proline recognition, employed by WW domains and SH3 domains.
Disease
Known diseases associated with this structure: Becker muscular dystrophy OMIM:[300377], Cardiomyopathy, dilated, 3B OMIM:[300377], Duchenne muscular dystrophy OMIM:[300377]
About this Structure
1EG4 is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Structure of a WW domain containing fragment of dystrophin in complex with beta-dystroglycan., Huang X, Poy F, Zhang R, Joachimiak A, Sudol M, Eck MJ, Nat Struct Biol. 2000 Aug;7(8):634-8. PMID:10932245
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