1ijb
From Proteopedia
(New page: 200px<br /> <applet load="1ijb" size="450" color="white" frame="true" align="right" spinBox="true" caption="1ijb, resolution 1.80Å" /> '''The von Willebrand ...) |
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caption="1ijb, resolution 1.80Å" /> | caption="1ijb, resolution 1.80Å" /> | ||
'''The von Willebrand Factor mutant (I546V) A1 domain'''<br /> | '''The von Willebrand Factor mutant (I546V) A1 domain'''<br /> | ||
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==About this Structure== | ==About this Structure== | ||
| - | 1IJB is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http:// | + | 1IJB is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence 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=1IJB OCA]. |
==Reference== | ==Reference== | ||
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[[Category: dinucleotide-binding fold]] | [[Category: dinucleotide-binding fold]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri Feb 15 16:01:39 2008'' |
Revision as of 14:01, 15 February 2008
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The von Willebrand Factor mutant (I546V) A1 domain
Contents |
Overview
The A1 domain of von Willebrand factor (vWF) mediates platelet adhesion to, sites of vascular injury by binding to the platelet receptor glycoprotein, Ib (GpIb), an interaction that is regulated by hydrodynamic shear forces., The GpIb binding surface of A1 is distinct from a regulatory region, suggesting that ligand binding is controlled allosterically. Here we, report the crystal structures of the "gain-of-function" mutant A1 domain, (I546V) and its complex with the exogenous activator botrocetin. We show, that botrocetin switches the mutant A1 back toward the wild-type, conformation, suggesting that affinity is enhanced by augmenting the GpIb, binding surface rather than through allosteric control. Functional studies, of platelet adhesion under flow further suggest that the activation, mechanism is distinct from that of the gain-of-function mutation.
Disease
Known diseases associated with this structure: von Willebrand disease OMIM:[193400]
About this Structure
1IJB is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Structural basis of von Willebrand factor activation by the snake toxin botrocetin., Fukuda K, Doggett TA, Bankston LA, Cruz MA, Diacovo TG, Liddington RC, Structure. 2002 Jul;10(7):943-50. PMID:12121649
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