View source for 1gjj


==N-TERMINAL CONSTANT REGION OF THE NUCLEAR ENVELOPE PROTEIN LAP2==
<StructureSection load='1gjj' size='340' side='right'caption='[[1gjj]], [[NMR_Ensembles_of_Models | 1 NMR models]]' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[1gjj]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1GJJ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1GJJ FirstGlance]. <br>
</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=1gjj FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1gjj OCA], [https://pdbe.org/1gjj PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1gjj RCSB], [https://www.ebi.ac.uk/pdbsum/1gjj PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1gjj ProSAT]</span></td></tr>
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== 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/gj/1gjj_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=1gjj ConSurf].
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== Publication Abstract from PubMed ==
The nuclear envelope proteins LAP2, emerin and MAN1 share a conserved approximately 40-residue 'LEM' motif. Loss of emerin causes Emery-Dreifuss muscular dystrophy. We have solved the solution NMR structure of the constant region of human LAP2 (residues 1-168). Human LAP2(1-168) has two structurally independent, non-interacting domains located at residues 1-50 ('LAP2-N') and residues 111-152 (LEM-domain), connected by an approximately 60-residue flexible linker. The two domains are structurally homologous, comprising a helical turn followed by two helices connected by an 11-12-residue loop. This motif is shared by subdomains of T4 endonuclease VII and transcription factor rho, despite negligible (&lt; or =15%) sequence identity. NMR chemical shift mapping demonstrated that the LEM-domain binds BAF (barrier-to-autointegration factor), whereas LAP2-N binds DNA. Both binding surfaces comprise helix 1, the N-terminus of helix 2 and the inter-helical loop. Binding selectivity is determined by the nature of the surface residues in these binding sites, which are predominantly positively charged for LAP2-N and hydrophobic for the LEM-domain. Thus, LEM and LEM-like motifs form a common structure that evolution has customized for binding to BAF or DNA.

Solution structure of the constant region of nuclear envelope protein LAP2 reveals two LEM-domain structures: one binds BAF and the other binds DNA.,Cai M, Huang Y, Ghirlando R, Wilson KL, Craigie R, Clore GM EMBO J. 2001 Aug 15;20(16):4399-407. PMID:11500367<ref>PMID:11500367</ref>

From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Human]]
[[Category: Large Structures]]
[[Category: Cai, M]]
[[Category: Clore, G M]]
[[Category: Inner nuclear membrane protein]]
[[Category: Lamin-associated polypeptide]]
[[Category: Lem domain]]
[[Category: Membrane protein]]
[[Category: Multidimensional nmr dipolar coupling]]