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1h8b

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[[Image:1h8b.gif|left|200px]]
 
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{{Structure
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==EF-hands 3,4 from alpha-actinin / Z-repeat 7 from titin==
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|PDB= 1h8b |SIZE=350|CAPTION= <scene name='initialview01'>1h8b</scene>
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<StructureSection load='1h8b' size='340' side='right'caption='[[1h8b]]' scene=''>
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|SITE=
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== Structural highlights ==
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|LIGAND=
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<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>
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|ACTIVITY=
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
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|GENE=
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<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>
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}}
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</table>
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== Disease ==
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[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>
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== Function ==
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[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.
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/h8/1h8b_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</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].
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<div style="clear:both"></div>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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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.
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'''EF-HANDS 3,4 FROM ALPHA-ACTININ / Z-REPEAT 7 FROM TITIN'''
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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>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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<div class="pdbe-citations 1h8b" style="background-color:#fffaf0;"></div>
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==Overview==
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==See Also==
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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.
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*[[Actinin 3D structures|Actinin 3D structures]]
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*[[Titin 3D structures|Titin 3D structures]]
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==About this Structure==
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== References ==
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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].
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<references/>
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__TOC__
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==Reference==
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</StructureSection>
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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]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Large Structures]]
[[Category: Oryctolagus cuniculus]]
[[Category: Oryctolagus cuniculus]]
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[[Category: Protein complex]]
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[[Category: Atkinson RA]]
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[[Category: Atkinson, R A.]]
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[[Category: Frenkiel TA]]
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[[Category: Frenkiel, T A.]]
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[[Category: Joseph C]]
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[[Category: Joseph, C.]]
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[[Category: Kelly G]]
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[[Category: Kelly, G.]]
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[[Category: Muskett FW]]
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[[Category: Muskett, F W.]]
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[[Category: Nietlispach D]]
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[[Category: Nietlispach, D.]]
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[[Category: Pastore A]]
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[[Category: Pastore, A.]]
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[[Category: z-disk structural complex]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 11:33:38 2008''
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Current revision

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

PDB ID 1h8b

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