1emo: Difference between revisions
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[[Image:1emo.gif|left|200px]]<br /> | [[Image:1emo.gif|left|200px]]<br /><applet load="1emo" size="450" color="white" frame="true" align="right" spinBox="true" | ||
<applet load="1emo" size="450" color="white" frame="true" align="right" spinBox="true" | |||
caption="1emo" /> | caption="1emo" /> | ||
'''NMR STUDY OF A PAIR OF FIBRILLIN CA2+ BINDING EPIDERMAL GROWTH FACTOR-LIKE DOMAINS, 22 STRUCTURES'''<br /> | '''NMR STUDY OF A PAIR OF FIBRILLIN CA2+ BINDING EPIDERMAL GROWTH FACTOR-LIKE DOMAINS, 22 STRUCTURES'''<br /> | ||
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==About this Structure== | ==About this Structure== | ||
1EMO is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with CA as [http://en.wikipedia.org/wiki/ligand ligand]. | 1EMO is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with CA as [http://en.wikipedia.org/wiki/ligand ligand]. Known structural/functional Sites: <scene name='pdbsite=CA1:Ca Binding Site For Egf-Like Domain 32'>CA1</scene> and <scene name='pdbsite=CA2:Ca Binding Site For Egf-Like Domain 33'>CA2</scene>. Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=1EMO OCA]. | ||
==Reference== | ==Reference== | ||
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[[Category: signal]] | [[Category: signal]] | ||
''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on | ''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Tue Dec 18 15:13:34 2007'' | ||
Revision as of 16:03, 18 December 2007
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NMR STUDY OF A PAIR OF FIBRILLIN CA2+ BINDING EPIDERMAL GROWTH FACTOR-LIKE DOMAINS, 22 STRUCTURES
OverviewOverview
The nuclear magnetic resonance structure of a covalently linked pair of, calcium-binding (cb) epidermal growth factor-like (EGF) domains from human, fibrillin-1, the protein defective in the Marfan syndrome, is described., The two domains are in a rigid, rod-like arrangement, stabilized by, interdomain calcium binding and hydrophobic interactions. We propose a, model for the arrangement of fibrillin monomers in microfibrils that, reconciles structural and antibody binding data, and we describe a set of, disease-causing mutations that provide the first clues to the specificity, of cbEFG interactions. The residues involved in stabilizing the domain, linkage are highly conserved in fibrillin, fibulin, thrombomodulin, and, the low density lipoprotein receptor. We propose that the relative, orientation of tandem cbEGF domains in these proteins is similar, but that, in others, including Notch, pairs adopt a completely different, conformation.
DiseaseDisease
Known diseases associated with this structure: Aortic aneurysm, ascending, and dissection OMIM:[134797], Ectopia lentis, familial OMIM:[134797], MASS syndrome OMIM:[134797], Marfan syndrome OMIM:[134797], Shprintzen-Goldberg syndrome OMIM:[134797], Weill-Marchesani syndrome, dominant OMIM:[134797]
About this StructureAbout this Structure
1EMO is a Single protein structure of sequence from Homo sapiens with CA as ligand. Known structural/functional Sites: and . Full crystallographic information is available from OCA.
ReferenceReference
Solution structure of a pair of calcium-binding epidermal growth factor-like domains: implications for the Marfan syndrome and other genetic disorders., Downing AK, Knott V, Werner JM, Cardy CM, Campbell ID, Handford PA, Cell. 1996 May 17;85(4):597-605. PMID:8653794
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