1rgw

From Proteopedia

(Difference between revisions)
Jump to: navigation, search
Line 1: Line 1:
-
{{STRUCTURE_1rgw| PDB=1rgw | SCENE= }}
+
==Solution Structure of ZASP's PDZ domain==
-
===Solution Structure of ZASP's PDZ domain===
+
<StructureSection load='1rgw' size='340' side='right' caption='[[1rgw]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''>
-
{{ABSTRACT_PUBMED_15062084}}
+
== Structural highlights ==
 +
<table><tr><td colspan='2'>[[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]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1RGW FirstGlance]. <br>
 +
</td></tr><tr><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">ZASP ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
 +
<tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1rgw FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1rgw OCA], [http://www.rcsb.org/pdb/explore.do?structureId=1rgw RCSB], [http://www.ebi.ac.uk/pdbsum/1rgw PDBsum]</span></td></tr>
 +
<table>
 +
== Disease ==
 +
[[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.
 +
== Function ==
 +
[[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.[:]
 +
== 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/rg/1rgw_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/chain_selection.php?pdb_ID=2ata ConSurf].
 +
<div style="clear:both"></div>
 +
<div style="background-color:#fffaf0;">
 +
== Publication Abstract from PubMed ==
 +
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.
-
==Disease==
+
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>
-
[[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.
+
-
==Function==
+
From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
-
[[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.[:]
+
</div>
-
 
+
-
==About this Structure==
+
-
[[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].
+
==See Also==
==See Also==
*[[Group:MUZIC:ZASP|MUZIC:ZASP]]
*[[Group:MUZIC:ZASP|MUZIC:ZASP]]
*[[ZASP protein|ZASP protein]]
*[[ZASP protein|ZASP protein]]
-
 
+
== References ==
-
==Reference==
+
<references/>
-
<ref group="xtra">PMID:015062084</ref><references group="xtra"/><references/>
+
__TOC__
 +
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Atkinson, R A.]]
[[Category: Atkinson, R A.]]

Revision as of 16:03, 29 September 2014

Solution Structure of ZASP's PDZ domain

Drag the structure with the mouse to rotate

Proteopedia Page Contributors and Editors (what is this?)

OCA

Views
Personal tools
Navigation
Toolbox