1rgw: Difference between revisions

No edit summary
No edit summary
 
(14 intermediate revisions by the same user not shown)
Line 1: Line 1:
[[Image:1rgw.gif|left|200px]]


{{Structure
==Solution Structure of ZASP's PDZ domain==
|PDB= 1rgw |SIZE=350|CAPTION= <scene name='initialview01'>1rgw</scene>
<StructureSection load='1rgw' size='340' side='right'caption='[[1rgw]]' scene=''>
|SITE=  
== Structural highlights ==
|LIGAND=  
<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>
|ACTIVITY=  
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
|GENE= ZASP ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
<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>
|DOMAIN=
</table>
|RELATEDENTRY=
== Disease ==
|RESOURCES=<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.ebi.ac.uk/pdbsum/1rgw PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1rgw RCSB]</span>
[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.
}}
== Function ==
 
[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.[:]
'''Solution Structure of ZASP's PDZ domain'''
== Evolutionary Conservation ==
 
[[Image:Consurf_key_small.gif|200px|right]]
 
Check<jmol>
==Overview==
  <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/main_output.php?pdb_ID=1rgw 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.
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>
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]]


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


==Reference==
==See Also==
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]
*[[PDZ and LIM domain protein|PDZ and LIM domain protein]]
*[[ZASP protein|ZASP protein]]
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Single protein]]
[[Category: Large Structures]]
[[Category: Atkinson, R A.]]
[[Category: Atkinson RA]]
[[Category: Au, Y.]]
[[Category: Au Y]]
[[Category: Faulkner, G.]]
[[Category: Faulkner G]]
[[Category: Guerrini, R.]]
[[Category: Guerrini R]]
[[Category: Joseph, C.]]
[[Category: Joseph C]]
[[Category: Martin, S R.]]
[[Category: Martin SR]]
[[Category: Muskett, F W.]]
[[Category: Muskett FW]]
[[Category: Pallavicini, A.]]
[[Category: Pallavicini A]]
[[Category: Pastore, A.]]
[[Category: Pastore A]]
[[Category: cypher]]
[[Category: muscle]]
[[Category: oracle]]
[[Category: pdz]]
[[Category: sarcomere]]
[[Category: z-disk]]
[[Category: zasp]]
 
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 23:28:00 2008''

Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)

OCA