2aav
From Proteopedia
(New page: 200px<br /> <applet load="2aav" size="450" color="white" frame="true" align="right" spinBox="true" caption="2aav" /> '''Solution NMR structure of Filamin A domain ...) |
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'''Solution NMR structure of Filamin A domain 17'''<br /> | '''Solution NMR structure of Filamin A domain 17'''<br /> | ||
==Overview== | ==Overview== | ||
| - | Filamin A (FLNa), a dimeric actin cross-linking and scaffold protein with | + | 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. |
==Disease== | ==Disease== | ||
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==About this Structure== | ==About this Structure== | ||
| - | 2AAV 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:// | + | 2AAV 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=2AAV OCA]. |
==Reference== | ==Reference== | ||
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[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Single protein]] | [[Category: Single protein]] | ||
| - | [[Category: Hartwig, J | + | [[Category: Hartwig, J H.]] |
[[Category: Heikkinen, O.]] | [[Category: Heikkinen, O.]] | ||
[[Category: Kilpelainen, I.]] | [[Category: Kilpelainen, I.]] | ||
| - | [[Category: Munday, A | + | [[Category: Munday, A D.]] |
[[Category: Nakamura, F.]] | [[Category: Nakamura, F.]] | ||
[[Category: Permi, P.]] | [[Category: Permi, P.]] | ||
[[Category: Pudas, R.]] | [[Category: Pudas, R.]] | ||
| - | [[Category: Stossel, T | + | [[Category: Stossel, T P.]] |
[[Category: Ylanne, J.]] | [[Category: Ylanne, J.]] | ||
[[Category: beta-sandwich]] | [[Category: beta-sandwich]] | ||
[[Category: filamin a domain 17]] | [[Category: filamin a domain 17]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 16:25:36 2008'' |
Revision as of 14:25, 21 February 2008
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Solution NMR structure of Filamin A domain 17
Contents |
Overview
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.
Disease
Known diseases associated with this structure: 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], Otopalatodigital syndrome, type I OMIM:[300017], Otopalatodigital syndrome, type II OMIM:[300017]
About this Structure
2AAV is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
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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