1rgw

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{{Seed}}
 
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[[Image:1rgw.png|left|200px]]
 
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==Solution Structure of ZASP's PDZ domain==
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The line below this paragraph, containing "STRUCTURE_1rgw", creates the "Structure Box" on the page.
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<StructureSection load='1rgw' size='340' side='right'caption='[[1rgw]]' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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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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or leave the SCENE parameter empty for the default display.
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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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{{STRUCTURE_1rgw| PDB=1rgw | SCENE= }}
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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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===Solution Structure of ZASP's PDZ domain===
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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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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 1rgw" style="background-color:#fffaf0;"></div>
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==See Also==
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The line below this paragraph, {{ABSTRACT_PUBMED_15062084}}, adds the Publication Abstract to the page
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*[[PDZ and LIM domain protein|PDZ and LIM domain protein]]
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(as it appears on PubMed at http://www.pubmed.gov), where 15062084 is the PubMed ID number.
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*[[ZASP protein|ZASP protein]]
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== References ==
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{{ABSTRACT_PUBMED_15062084}}
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<references/>
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__TOC__
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==Disease==
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</StructureSection>
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Known disease associated with this structure: Cardiomyopathy, dilated OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=605906 605906]], Cardiomyopathy, dilated, with left ventricular noncompaction OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=605906 605906]], Myopathy, myofibrillar, ZASP-related OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=605906 605906]]
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==About this Structure==
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1RGW is a [[Single protein]] structure of 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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==Reference==
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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:[http://www.ncbi.nlm.nih.gov/pubmed/15062084 15062084]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Atkinson, R A.]]
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[[Category: Atkinson RA]]
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[[Category: Au, Y.]]
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[[Category: Au Y]]
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[[Category: Faulkner, G.]]
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[[Category: Faulkner G]]
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[[Category: Guerrini, R.]]
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[[Category: Guerrini R]]
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[[Category: Joseph, C.]]
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[[Category: Joseph C]]
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[[Category: Martin, S R.]]
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[[Category: Martin SR]]
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[[Category: Muskett, F W.]]
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[[Category: Muskett FW]]
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[[Category: Pallavicini, A.]]
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[[Category: Pallavicini A]]
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[[Category: Pastore, A.]]
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[[Category: Pastore A]]
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[[Category: Cypher]]
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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: Z-disk]]
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[[Category: Zasp]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 29 14:03:28 2008''
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Current revision

Solution Structure of ZASP's PDZ domain

PDB ID 1rgw

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