1rgw

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{{STRUCTURE_1rgw| PDB=1rgw | SCENE= }}
 
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===Solution Structure of ZASP's PDZ domain===
 
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{{ABSTRACT_PUBMED_15062084}}
 
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==Disease==
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==Solution Structure of ZASP's PDZ domain==
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[[http://www.uniprot.org/uniprot/LDB3_HUMAN LDB3_HUMAN]] Defects in LDB3 are the cause of cardiomyopathy dilated type 1C (CMD1C) [MIM:[http://omim.org/entry/601493 601493]]. 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:14662268</ref><ref>PMID:14660611</ref> Defects in LDB3 are the cause of left ventricular non-compaction type 3 (LVNC3) [MIM:[http://omim.org/entry/601493 601493]]. Left ventricular non-compaction is characterized by numerous prominent trabeculations and deep intertrabecular recesses in hypertrophied and hypokinetic segments of the left ventricle. Defects in LDB3 are the cause of myopathy myofibrillar type 4 (MFM4) [MIM:[http://omim.org/entry/609452 609452]]. A neuromuscular disorder characterized by distal and proximal muscle weakness with signs of cardiomyopathy and neuropathy.
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<StructureSection load='1rgw' size='340' side='right'caption='[[1rgw]]' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[1rgw]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1RGW OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1RGW FirstGlance]. <br>
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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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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1rgw FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1rgw OCA], [https://pdbe.org/1rgw PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1rgw RCSB], [https://www.ebi.ac.uk/pdbsum/1rgw PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1rgw ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/LDB3_HUMAN LDB3_HUMAN] Defects in LDB3 are the cause of cardiomyopathy dilated type 1C (CMD1C) [MIM:[https://omim.org/entry/601493 601493]. 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:14662268</ref> <ref>PMID:14660611</ref> Defects in LDB3 are the cause of left ventricular non-compaction type 3 (LVNC3) [MIM:[https://omim.org/entry/601493 601493]. Left ventricular non-compaction is characterized by numerous prominent trabeculations and deep intertrabecular recesses in hypertrophied and hypokinetic segments of the left ventricle. Defects in LDB3 are the cause of myopathy myofibrillar type 4 (MFM4) [MIM:[https://omim.org/entry/609452 609452]. A neuromuscular disorder characterized by distal and proximal muscle weakness with signs of cardiomyopathy and neuropathy.
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== Function ==
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[https://www.uniprot.org/uniprot/LDB3_HUMAN LDB3_HUMAN] May function as an adapter in striated muscle to couple protein kinase C-mediated signaling via its LIM domains to the cytoskeleton.[:]
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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/rg/1rgw_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=1rgw 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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Z band alternately spliced PDZ-containing protein (ZASP) is a sarcomere Z disk protein expressed in human cardiac and skeletal muscle that is thought to be involved in a dominant familial dilated cardiomyopathy. The N-terminal PDZ domain of ZASP interacts with the C terminus of alpha-actinin-2, the major component of the Z disk, probably by forming a ternary complex with titin Z repeats. We have determined the structure of ZASP PDZ by NMR and showed that it is a classical class 1 PDZ domain that recognizes the carboxy-terminal sequence of an alpha-actinin-2 calmodulin-like domain with micromolar affinity. We also characterized the role of each component in the ternary complex ZASP/alpha-actinin-2/titin, showing that the alpha-actinin-2/ZASP PDZ interaction involves a binding surface distinct from that recognized by the titin Z repeats. ZASP PDZ structure was used to model other members of the enigma family by homology and to predict their abilities to bind alpha-actinin-2.
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==Function==
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Solution structure of ZASP PDZ domain; implications for sarcomere ultrastructure and enigma family redundancy.,Au Y, Atkinson RA, Guerrini R, Kelly G, Joseph C, Martin SR, Muskett FW, Pallavicini A, Faulkner G, Pastore A Structure. 2004 Apr;12(4):611-22. PMID:15062084<ref>PMID:15062084</ref>
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[[http://www.uniprot.org/uniprot/LDB3_HUMAN LDB3_HUMAN]] May function as an adapter in striated muscle to couple protein kinase C-mediated signaling via its LIM domains to the cytoskeleton.[:]
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==About this Structure==
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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[[1rgw]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1RGW OCA].
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</div>
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<div class="pdbe-citations 1rgw" style="background-color:#fffaf0;"></div>
==See Also==
==See Also==
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*[[Group:MUZIC:ZASP|MUZIC:ZASP]]
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*[[PDZ and LIM domain protein|PDZ and LIM domain protein]]
*[[ZASP protein|ZASP protein]]
*[[ZASP protein|ZASP protein]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:015062084</ref><references group="xtra"/><references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Atkinson, R A.]]
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[[Category: Large Structures]]
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[[Category: Au, Y.]]
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[[Category: Atkinson RA]]
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[[Category: Faulkner, G.]]
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[[Category: Au Y]]
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[[Category: Guerrini, R.]]
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[[Category: Faulkner G]]
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[[Category: Joseph, C.]]
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[[Category: Guerrini R]]
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[[Category: Martin, S R.]]
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[[Category: Joseph C]]
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[[Category: Muskett, F W.]]
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[[Category: Martin SR]]
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[[Category: Pallavicini, A.]]
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[[Category: Muskett FW]]
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[[Category: Pastore, A.]]
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[[Category: Pallavicini A]]
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[[Category: Cypher]]
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[[Category: Pastore A]]
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[[Category: Muscle]]
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[[Category: Oracle]]
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[[Category: Pdz]]
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[[Category: Sarcomere]]
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[[Category: Structural protein]]
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[[Category: Z-disk]]
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[[Category: Zasp]]
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Current revision

Solution Structure of ZASP's PDZ domain

PDB ID 1rgw

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