1uzp
From Proteopedia
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|PDB= 1uzp |SIZE=350|CAPTION= <scene name='initialview01'>1uzp</scene>, resolution 1.78Å | |PDB= 1uzp |SIZE=350|CAPTION= <scene name='initialview01'>1uzp</scene>, resolution 1.78Å | ||
|SITE= <scene name='pdbsite=AC1:Sm+Binding+Site+For+Chain+A'>AC1</scene> | |SITE= <scene name='pdbsite=AC1:Sm+Binding+Site+For+Chain+A'>AC1</scene> | ||
| - | |LIGAND= <scene name='pdbligand=SM:SAMARIUM (III) ION'>SM</scene> | + | |LIGAND= <scene name='pdbligand=SM:SAMARIUM+(III)+ION'>SM</scene> |
|ACTIVITY= | |ACTIVITY= | ||
|GENE= | |GENE= | ||
| + | |DOMAIN= | ||
| + | |RELATEDENTRY= | ||
| + | |RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1uzp FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1uzp OCA], [http://www.ebi.ac.uk/pdbsum/1uzp PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1uzp RCSB]</span> | ||
}} | }} | ||
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==Overview== | ==Overview== | ||
Human fibrillin-1, the major structural protein of extracellular matrix (ECM) 10-12 nm microfibrils, is dominated by 43 calcium binding epidermal growth factor-like (cbEGF) and 7 transforming growth factor beta binding protein-like (TB) domains. Crystal structures reveal the integrin binding cbEGF22-TB4-cbEGF23 fragment of human fibrillin-1 to be a Ca(2+)-rigidified tetragonal pyramid. We suggest that other cbEGF-TB pairs within the fibrillins may adopt a similar orientation to cbEGF22-TB4. In addition, we have located a flexible RGD integrin binding loop within TB4. Modeling, cell attachment and spreading assays, immunocytochemistry, and surface plasmon resonance indicate that cbEGF22 bound to TB4 is a requirement for integrin activation and provide insight into the molecular basis of the fibrillin-1 interaction with alphaVbeta3. In light of our data, we propose a novel model for the assembly of the fibrillin microfibril and a mechanism to explain its extensibility. | Human fibrillin-1, the major structural protein of extracellular matrix (ECM) 10-12 nm microfibrils, is dominated by 43 calcium binding epidermal growth factor-like (cbEGF) and 7 transforming growth factor beta binding protein-like (TB) domains. Crystal structures reveal the integrin binding cbEGF22-TB4-cbEGF23 fragment of human fibrillin-1 to be a Ca(2+)-rigidified tetragonal pyramid. We suggest that other cbEGF-TB pairs within the fibrillins may adopt a similar orientation to cbEGF22-TB4. In addition, we have located a flexible RGD integrin binding loop within TB4. Modeling, cell attachment and spreading assays, immunocytochemistry, and surface plasmon resonance indicate that cbEGF22 bound to TB4 is a requirement for integrin activation and provide insight into the molecular basis of the fibrillin-1 interaction with alphaVbeta3. In light of our data, we propose a novel model for the assembly of the fibrillin microfibril and a mechanism to explain its extensibility. | ||
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| - | ==Disease== | ||
| - | Known diseases associated with this structure: Aortic aneurysm, ascending, and dissection OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=134797 134797]], Ectopia lentis, familial OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=134797 134797]], MASS syndrome OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=134797 134797]], Marfan syndrome OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=134797 134797]], Shprintzen-Goldberg syndrome OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=134797 134797]], Weill-Marchesani syndrome, dominant OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=134797 134797]] | ||
==About this Structure== | ==About this Structure== | ||
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[[Category: Lee, S S.J.]] | [[Category: Lee, S S.J.]] | ||
[[Category: Stuart, D I.]] | [[Category: Stuart, D I.]] | ||
| - | [[Category: SM]] | ||
[[Category: cbegf domain]] | [[Category: cbegf domain]] | ||
[[Category: extra-cellular matrix]] | [[Category: extra-cellular matrix]] | ||
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[[Category: tb domain]] | [[Category: tb domain]] | ||
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Mar 31 00:17:36 2008'' |
Revision as of 21:17, 30 March 2008
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| , resolution 1.78Å | |||||||
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| Resources: | FirstGlance, OCA, PDBsum, RCSB | ||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||
INTEGRIN BINDING CBEGF22-TB4-CBEGF33 FRAGMENT OF HUMAN FIBRILLIN-1, SM BOUND FORM CBEGF23 DOMAIN ONLY.
Overview
Human fibrillin-1, the major structural protein of extracellular matrix (ECM) 10-12 nm microfibrils, is dominated by 43 calcium binding epidermal growth factor-like (cbEGF) and 7 transforming growth factor beta binding protein-like (TB) domains. Crystal structures reveal the integrin binding cbEGF22-TB4-cbEGF23 fragment of human fibrillin-1 to be a Ca(2+)-rigidified tetragonal pyramid. We suggest that other cbEGF-TB pairs within the fibrillins may adopt a similar orientation to cbEGF22-TB4. In addition, we have located a flexible RGD integrin binding loop within TB4. Modeling, cell attachment and spreading assays, immunocytochemistry, and surface plasmon resonance indicate that cbEGF22 bound to TB4 is a requirement for integrin activation and provide insight into the molecular basis of the fibrillin-1 interaction with alphaVbeta3. In light of our data, we propose a novel model for the assembly of the fibrillin microfibril and a mechanism to explain its extensibility.
About this Structure
1UZP is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Structure of the integrin binding fragment from fibrillin-1 gives new insights into microfibril organization., Lee SS, Knott V, Jovanovic J, Harlos K, Grimes JM, Choulier L, Mardon HJ, Stuart DI, Handford PA, Structure. 2004 Apr;12(4):717-29. PMID:15062093
Page seeded by OCA on Mon Mar 31 00:17:36 2008
