2bp3: Difference between revisions
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[[Image:2bp3.gif|left|200px]]<br /> | [[Image:2bp3.gif|left|200px]]<br /><applet load="2bp3" size="450" color="white" frame="true" align="right" spinBox="true" | ||
<applet load="2bp3" size="450" color="white" frame="true" align="right" spinBox="true" | |||
caption="2bp3, resolution 2.32Å" /> | caption="2bp3, resolution 2.32Å" /> | ||
'''CRYSTAL STRUCTURE OF FILAMIN A DOMAIN 17 AND GPIB ALPHA CYTOPLASMIC DOMAIN COMPLEX'''<br /> | '''CRYSTAL STRUCTURE OF FILAMIN A DOMAIN 17 AND GPIB ALPHA CYTOPLASMIC DOMAIN COMPLEX'''<br /> | ||
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==About this Structure== | ==About this Structure== | ||
2BP3 is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with GOL as [http://en.wikipedia.org/wiki/ligand ligand]. | 2BP3 is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with GOL as [http://en.wikipedia.org/wiki/ligand ligand]. Known structural/functional Site: <scene name='pdbsite=AC1:Gol Binding Site For Chain A'>AC1</scene>. Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=2BP3 OCA]. | ||
==Reference== | ==Reference== | ||
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[[Category: cytoskeleton]] | [[Category: cytoskeleton]] | ||
''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 18:54:46 2007'' | ||
Revision as of 19:44, 18 December 2007
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CRYSTAL STRUCTURE OF FILAMIN A DOMAIN 17 AND GPIB ALPHA CYTOPLASMIC DOMAIN COMPLEX
OverviewOverview
Filamin A (FLNa), a dimeric actin cross-linking and scaffold protein with, numerous intracellular binding partners, anchors the platelet adhesion, glycoprotein (GP) Ib-IX-V receptor to actin cytoskeleton. We mapped the, GPIbalpha binding site to a single domain of FLNa and resolved the, structure of this domain and its interaction complex with the, corresponding GPIbalpha cytoplasmic domain. This is the first atomic, structure of this class of membrane glycoprotein-cytoskeleton connection., GPIbalpha binds in a groove formed between the C and D beta strands of, FLNa domain 17. The interaction is strikingly similar to that between the, beta7 integrin tail and a different FLNa domain, potentially defining a, conserved motif for FLNa binding. Nevertheless, the structures also reveal, specificity of the interfaces, which explains different regulatory, mechanisms. To verify the topology of GPIb-FLNa interaction we also, purified the native complex from platelets and showed that GPIb interacts, with the C-terminus of FLNa, which is in accordance with our biochemical, and structural data.
DiseaseDisease
Known diseases associated with this structure: Bernard-Soulier syndrome, type A OMIM:[606672], Frontometaphyseal dysplasia OMIM:[300017], Heterotopia, periventricular OMIM:[300017], Heterotopia, periventricular nodular, with frontometaphyseal dysplasia OMIM:[300017], Heterotopia, periventricular, ED variant OMIM:[300017], Melnick-Needles syndrome OMIM:[300017], Nonarteritic anterior ischemic optic neuropathy, susceptibility to OMIM:[606672], Otopalatodigital syndrome, type I OMIM:[300017], Otopalatodigital syndrome, type II OMIM:[300017], von Willebrand disease, platelet-type OMIM:[606672]
About this StructureAbout this Structure
2BP3 is a Protein complex structure of sequences from Homo sapiens with GOL as ligand. Known structural/functional Site: . Full crystallographic information is available from OCA.
ReferenceReference
The structure of the GPIb-filamin A complex., Nakamura F, Pudas R, Heikkinen O, Permi P, Kilpelainen I, Munday AD, Hartwig JH, Stossel TP, Ylanne J, Blood. 2006 Mar 1;107(5):1925-32. Epub 2005 Nov 17. PMID:16293600
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