3my0: Difference between revisions

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{{Large structure}}
==Crystal structure of the ACVRL1 (ALK1) kinase domain bound to LDN-193189==
{{STRUCTURE_3my0|  PDB=3my0  |  SCENE=  }}
<StructureSection load='3my0' size='340' side='right' caption='[[3my0]], [[Resolution|resolution]] 2.65&Aring;' scene=''>
===Crystal structure of the ACVRL1 (ALK1) kinase domain bound to LDN-193189===
== Structural highlights ==
 
<table><tr><td colspan='2'>[[3my0]] is a 24 chain structure with 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=3MY0 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3MY0 FirstGlance]. <br>
==About this Structure==
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=LDN:4-[6-(4-PIPERAZIN-1-YLPHENYL)PYRAZOLO[1,5-A]PYRIMIDIN-3-YL]QUINOLINE'>LDN</scene></td></tr>
[[3my0]] is a 24 chain structure with 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=3MY0 OCA].  
<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">ACVRL1, ACVRL1 (ALK1), ACVRLK1, ALK1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Receptor_protein_serine/threonine_kinase Receptor protein serine/threonine kinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.11.30 2.7.11.30] </span></td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3my0 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3my0 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3my0 RCSB], [http://www.ebi.ac.uk/pdbsum/3my0 PDBsum]</span></td></tr>
</table>
== Disease ==
[[http://www.uniprot.org/uniprot/ACVL1_HUMAN ACVL1_HUMAN]] Defects in ACVRL1 are the cause of hereditary hemorrhagic telangiectasia type 2 (HHT2) [MIM:[http://omim.org/entry/600376 600376]]; also known as Osler-Rendu-Weber syndrome 2 (ORW2). HHT2 is an autosomal dominant multisystemic vascular dysplasia, characterized by recurrent epistaxis, muco-cutaneous telangiectases, gastro-intestinal hemorrhage, and pulmonary, cerebral and hepatic arteriovenous malformations; all secondary manifestations of the underlying vascular dysplasia.<ref>PMID:9245985</ref> <ref>PMID:8640225</ref> <ref>PMID:10694922</ref> <ref>PMID:10767348</ref> <ref>PMID:11170071</ref> <ref>PMID:11484689</ref> <ref>PMID:14684682</ref> <ref>PMID:15024723</ref> <ref>PMID:15712270</ref> 
== Function ==
[[http://www.uniprot.org/uniprot/ACVL1_HUMAN ACVL1_HUMAN]] Type I receptor for TGF-beta family ligands BMP9/GDF2 and BMP10 and important regulator of normal blood vessel development. On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. May bind activin as well.<ref>PMID:22799562</ref> <ref>PMID:22718755</ref> 
== 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/my/3my0_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>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Receptor protein serine/threonine kinase]]
[[Category: Receptor protein serine/threonine kinase]]
[[Category: Alfano, I.]]
[[Category: Alfano, I]]
[[Category: Arrowsmith, C H.]]
[[Category: Arrowsmith, C H]]
[[Category: Bountra, C.]]
[[Category: Bountra, C]]
[[Category: Bullock, A.]]
[[Category: Bullock, A]]
[[Category: Carpenter, C P.]]
[[Category: Carpenter, C P]]
[[Category: Chaikuad, A.]]
[[Category: Chaikuad, A]]
[[Category: Cooper, C.]]
[[Category: Cooper, C]]
[[Category: Daga, N.]]
[[Category: Daga, N]]
[[Category: Delft, F von.]]
[[Category: Delft, F von]]
[[Category: Edwards, A M.]]
[[Category: Edwards, A M]]
[[Category: Fedorov, O.]]
[[Category: Fedorov, O]]
[[Category: Gileadi, O.]]
[[Category: Gileadi, O]]
[[Category: Knapp, S.]]
[[Category: Knapp, S]]
[[Category: Krojer, T.]]
[[Category: Krojer, T]]
[[Category: Mahajan, P.]]
[[Category: Mahajan, P]]
[[Category: Muniz, J R.C.]]
[[Category: Muniz, J R.C]]
[[Category: Petrie, K.]]
[[Category: Petrie, K]]
[[Category: Pike, A C.W.]]
[[Category: Pike, A C.W]]
[[Category: SGC, Structural Genomics Consortium.]]
[[Category: Structural genomic]]
[[Category: Sanvitale, C.]]
[[Category: Sanvitale, C]]
[[Category: Savitsky, P.]]
[[Category: Savitsky, P]]
[[Category: Sethi, R.]]
[[Category: Sethi, R]]
[[Category: Ugochukwu, E.]]
[[Category: Ugochukwu, E]]
[[Category: Vollmar, M.]]
[[Category: Vollmar, M]]
[[Category: Weigelt, J.]]
[[Category: Weigelt, J]]
[[Category: Protein kinase]]
[[Category: Protein kinase]]
[[Category: Serine/threonine-protein kinase receptor]]
[[Category: Serine/threonine-protein kinase receptor]]
[[Category: Sgc]]
[[Category: Sgc]]
[[Category: Structural genomic]]
[[Category: Structural genomics consortium]]
[[Category: Transferase]]
[[Category: Transferase]]