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[[Image:1h8b.gif|left|200px]]
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{{STRUCTURE_1h8b|  PDB=1h8b  |  SCENE=  }}
'''EF-HANDS 3,4 FROM ALPHA-ACTININ / Z-REPEAT 7 FROM TITIN'''


==EF-hands 3,4 from alpha-actinin / Z-repeat 7 from titin==
<StructureSection load='1h8b' size='340' side='right'caption='[[1h8b]]' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[1h8b]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [https://en.wikipedia.org/wiki/Oryctolagus_cuniculus Oryctolagus cuniculus]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1H8B OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1H8B FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1h8b FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1h8b OCA], [https://pdbe.org/1h8b PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1h8b RCSB], [https://www.ebi.ac.uk/pdbsum/1h8b PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1h8b ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/ACTN2_HUMAN ACTN2_HUMAN] Defects in ACTN2 are the cause of cardiomyopathy dilated type 1AA (CMD1AA) [MIM:[https://omim.org/entry/612158 612158]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.<ref>PMID:14567970</ref>
== Function ==
[https://www.uniprot.org/uniprot/ACTN2_HUMAN ACTN2_HUMAN] F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein.
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
  <jmolCheckbox>
    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/h8/1h8b_consurf.spt"</scriptWhenChecked>
    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
    <text>to colour the structure by Evolutionary Conservation</text>
  </jmolCheckbox>
</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=1h8b ConSurf].
<div style="clear:both"></div>
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
The interaction between alpha-actinin and titin, two modular muscle proteins, is essential for sarcomere assembly. We have solved the solution structure of a complex between the calcium-insensitive C-terminal EF-hand domain of alpha-actinin-2 and the seventh Z-repeat of titin. The structure of the complex is in a semi-open conformation and closely resembles that of myosin light chains in their complexes with heavy chain IQ motifs. However, no IQ motif is present in the Z-repeat, suggesting that the semi-open conformation is a general structural solution for calcium-independent recognition of EF-hand domains.


==Overview==
Ca2+-independent binding of an EF-hand domain to a novel motif in the alpha-actinin-titin complex.,Atkinson RA, Joseph C, Kelly G, Muskett FW, Frenkiel TA, Nietlispach D, Pastore A Nat Struct Biol. 2001 Oct;8(10):853-7. PMID:11573089<ref>PMID:11573089</ref>
The interaction between alpha-actinin and titin, two modular muscle proteins, is essential for sarcomere assembly. We have solved the solution structure of a complex between the calcium-insensitive C-terminal EF-hand domain of alpha-actinin-2 and the seventh Z-repeat of titin. The structure of the complex is in a semi-open conformation and closely resembles that of myosin light chains in their complexes with heavy chain IQ motifs. However, no IQ motif is present in the Z-repeat, suggesting that the semi-open conformation is a general structural solution for calcium-independent recognition of EF-hand domains.


==About this Structure==
From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
1H8B is a [[Protein complex]] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [http://en.wikipedia.org/wiki/Oryctolagus_cuniculus Oryctolagus cuniculus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1H8B OCA].
</div>
<div class="pdbe-citations 1h8b" style="background-color:#fffaf0;"></div>


==Reference==
==See Also==
Ca2+-independent binding of an EF-hand domain to a novel motif in the alpha-actinin-titin complex., Atkinson RA, Joseph C, Kelly G, Muskett FW, Frenkiel TA, Nietlispach D, Pastore A, Nat Struct Biol. 2001 Oct;8(10):853-7. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/11573089 11573089]
*[[Actinin 3D structures|Actinin 3D structures]]
*[[Titin 3D structures|Titin 3D structures]]
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Oryctolagus cuniculus]]
[[Category: Oryctolagus cuniculus]]
[[Category: Protein complex]]
[[Category: Atkinson RA]]
[[Category: Atkinson, R A.]]
[[Category: Frenkiel TA]]
[[Category: Frenkiel, T A.]]
[[Category: Joseph C]]
[[Category: Joseph, C.]]
[[Category: Kelly G]]
[[Category: Kelly, G.]]
[[Category: Muskett FW]]
[[Category: Muskett, F W.]]
[[Category: Nietlispach D]]
[[Category: Nietlispach, D.]]
[[Category: Pastore A]]
[[Category: Pastore, A.]]
[[Category: Z-disk structural complex]]
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May  2 18:33:49 2008''

Latest revision as of 05:31, 15 May 2024

EF-hands 3,4 from alpha-actinin / Z-repeat 7 from titin

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