3my0: Difference between revisions
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==Crystal structure of the ACVRL1 (ALK1) kinase domain bound to LDN-193189== | |||
<StructureSection load='3my0' size='340' side='right' caption='[[3my0]], [[Resolution|resolution]] 2.65Å' scene=''> | |||
== 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> | |||
== | </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: | [[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: Transferase]] | [[Category: Transferase]] | ||