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[[Image:1h9e.jpg|left|200px]]<br /><applet load="1h9e" size="350" color="white" frame="true" align="right" spinBox="true"
caption="1h9e" />
'''LEM-LIKE DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN LAP2'''<br />


==Overview==
==LEM-LIKE DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN LAP2==
BACKGROUND: Integral membrane proteins of the inner nuclear membrane are, involved in chromatin organization and postmitotic reassembly of the, nucleus. The discovery that mutations in the gene encoding emerin causes, X-linked Emery-Dreifuss muscular dystrophy has enhanced interest in such, proteins. A common structural domain of 50 residues, called the LEM, domain, has been identified in emerin MAN1, and lamina-associated, polypeptide (LAP) 2. In particular, all LAP2 isoforms share an N-terminal, segment composed of such a LEM domain that is connected to a highly, divergent LEM-like domain by a linker that is probably unstructured., RESULTS: We have determined the three-dimensional structures of the LEM, and LEM-like domains of LAP2 using nuclear magnetic resonance and, molecular modeling. Both domains adopt the same fold, mainly composed of, two large parallel alpha helices. CONCLUSIONS: The structural LEM motif is, found in human inner nuclear membrane proteins and in protein-protein, interaction domains from bacterial multienzyme complexes. This suggests, that LEM and LEM-like domains are protein-protein interaction domains. A, region conserved in all LEM domains, at the surface of helix 2, could, mediate interaction between LEM domains and a common protein partner.
<StructureSection load='1h9e' size='340' side='right'caption='[[1h9e]]' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[1h9e]] 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=1H9E OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1H9E FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1h9e FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1h9e OCA], [https://pdbe.org/1h9e PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1h9e RCSB], [https://www.ebi.ac.uk/pdbsum/1h9e PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1h9e ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/LAP2A_HUMAN LAP2A_HUMAN] Defects in TMPO are the cause of cardiomyopathy dilated type 1T (CMD1T) [MIM:[https://omim.org/entry/613740 613740]. 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:16247757</ref>
== Function ==
[https://www.uniprot.org/uniprot/LAP2A_HUMAN LAP2A_HUMAN] May be involved in the structural organization of the nucleus and in the post-mitotic nuclear assembly. Plays an important role, together with LMNA, in the nuclear anchorage of RB1.  TP and TP5 may play a role in T-cell development and function. TP5 is an immunomodulating pentapeptide.
== 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/h9/1h9e_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=1h9e ConSurf].
<div style="clear:both"></div>
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
BACKGROUND: Integral membrane proteins of the inner nuclear membrane are involved in chromatin organization and postmitotic reassembly of the nucleus. The discovery that mutations in the gene encoding emerin causes X-linked Emery-Dreifuss muscular dystrophy has enhanced interest in such proteins. A common structural domain of 50 residues, called the LEM domain, has been identified in emerin MAN1, and lamina-associated polypeptide (LAP) 2. In particular, all LAP2 isoforms share an N-terminal segment composed of such a LEM domain that is connected to a highly divergent LEM-like domain by a linker that is probably unstructured. RESULTS: We have determined the three-dimensional structures of the LEM and LEM-like domains of LAP2 using nuclear magnetic resonance and molecular modeling. Both domains adopt the same fold, mainly composed of two large parallel alpha helices. CONCLUSIONS: The structural LEM motif is found in human inner nuclear membrane proteins and in protein-protein interaction domains from bacterial multienzyme complexes. This suggests that LEM and LEM-like domains are protein-protein interaction domains. A region conserved in all LEM domains, at the surface of helix 2, could mediate interaction between LEM domains and a common protein partner.


==Disease==
Structural characterization of the LEM motif common to three human inner nuclear membrane proteins.,Laguri C, Gilquin B, Wolff N, Romi-Lebrun R, Courchay K, Callebaut I, Worman HJ, Zinn-Justin S Structure. 2001 Jun;9(6):503-11. PMID:11435115<ref>PMID:11435115</ref>
Known disease associated with this structure: Cardiomyopathy, dilated, 1T OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188380 188380]]


==About this Structure==
From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
1H9E is a [http://en.wikipedia.org/wiki/Single_protein 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=1H9E OCA].
</div>
 
<div class="pdbe-citations 1h9e" style="background-color:#fffaf0;"></div>
==Reference==
== References ==
Structural characterization of the LEM motif common to three human inner nuclear membrane proteins., Laguri C, Gilquin B, Wolff N, Romi-Lebrun R, Courchay K, Callebaut I, Worman HJ, Zinn-Justin S, Structure. 2001 Jun;9(6):503-11. PMID:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=11435115 11435115]
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Single protein]]
[[Category: Large Structures]]
[[Category: Callebaut, I.]]
[[Category: Callebaut I]]
[[Category: Courchay, K.]]
[[Category: Courchay K]]
[[Category: Gilquin, B.]]
[[Category: Gilquin B]]
[[Category: Laguri, C.]]
[[Category: Laguri C]]
[[Category: Romi-Lebrun, R.]]
[[Category: Romi-Lebrun R]]
[[Category: Wolff, N.]]
[[Category: Wolff N]]
[[Category: Worman, H.J.]]
[[Category: Worman HJ]]
[[Category: Zinn-Justin, S.]]
[[Category: Zinn-Justin S]]
[[Category: emerin]]
[[Category: inner nuclear membrane protein]]
[[Category: lamina-associated polypeptide]]
[[Category: lem domain]]
[[Category: nmr]]
 
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri Feb 15 15:55:22 2008''

Latest revision as of 05:31, 15 May 2024

LEM-LIKE DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN LAP2

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