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3q4u

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{{STRUCTURE_3q4u| PDB=3q4u | SCENE= }}
 
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===Crystal structure of the ACVR1 kinase domain in complex with LDN-193189===
 
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==Disease==
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==Crystal structure of the ACVR1 kinase domain in complex with LDN-193189==
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[[http://www.uniprot.org/uniprot/ACVR1_HUMAN ACVR1_HUMAN]] Fibrodysplasia ossificans progressiva. Defects in ACVR1 are a cause of fibrodysplasia ossificans progressiva (FOP) [MIM:[http://omim.org/entry/135100 135100]]. FOP is a rare autosomal dominant disorder of skeletal malformations and progressive extraskeletal ossification. Heterotopic ossification in FOP begins in childhood and can be induced by trauma or may occur without warning. Bone formation is episodic and progressive, leading to extra-articular ankylosis of all major joints of the axial and appendicular skeleton, rendering movement impossible.<ref>PMID:16642017</ref> <ref>PMID:19085907</ref> <ref>PMID:19330033</ref>
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<StructureSection load='3q4u' size='340' side='right'caption='[[3q4u]], [[Resolution|resolution]] 1.82&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[3q4u]] is a 4 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3Q4U OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3Q4U FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/ACVR1_HUMAN ACVR1_HUMAN]] 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. Receptor for activin. May be involved for left-right pattern formation during embryogenesis (By similarity).
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.82&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=FLC:CITRATE+ANION'>FLC</scene>, <scene name='pdbligand=LDN:4-[6-(4-PIPERAZIN-1-YLPHENYL)PYRAZOLO[1,5-A]PYRIMIDIN-3-YL]QUINOLINE'>LDN</scene></td></tr>
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==About this Structure==
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3q4u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3q4u OCA], [https://pdbe.org/3q4u PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3q4u RCSB], [https://www.ebi.ac.uk/pdbsum/3q4u PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3q4u ProSAT]</span></td></tr>
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[[3q4u]] is a 4 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=3Q4U OCA].
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</table>
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== Disease ==
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==Reference==
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[https://www.uniprot.org/uniprot/ACVR1_HUMAN ACVR1_HUMAN] Fibrodysplasia ossificans progressiva. Defects in ACVR1 are a cause of fibrodysplasia ossificans progressiva (FOP) [MIM:[https://omim.org/entry/135100 135100]. FOP is a rare autosomal dominant disorder of skeletal malformations and progressive extraskeletal ossification. Heterotopic ossification in FOP begins in childhood and can be induced by trauma or may occur without warning. Bone formation is episodic and progressive, leading to extra-articular ankylosis of all major joints of the axial and appendicular skeleton, rendering movement impossible.<ref>PMID:16642017</ref> <ref>PMID:19085907</ref> <ref>PMID:19330033</ref>
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<references group="xtra"/><references/>
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== Function ==
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[https://www.uniprot.org/uniprot/ACVR1_HUMAN ACVR1_HUMAN] 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. Receptor for activin. May be involved for left-right pattern formation during embryogenesis (By similarity).
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Receptor protein serine/threonine kinase]]
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[[Category: Large Structures]]
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[[Category: Alfano, I.]]
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[[Category: Alfano I]]
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[[Category: Allerston, C K.]]
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[[Category: Allerston CK]]
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[[Category: Arrowsmith, C H.]]
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[[Category: Arrowsmith CH]]
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[[Category: Bountra, C.]]
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[[Category: Bountra C]]
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[[Category: Bullock, A.]]
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[[Category: Bullock A]]
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[[Category: Chaikuad, A.]]
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[[Category: Chaikuad A]]
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[[Category: Cooper, C D.O.]]
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[[Category: Cooper CDO]]
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[[Category: Daga, N.]]
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[[Category: Daga N]]
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[[Category: Delft, F von.]]
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[[Category: Edwards AM]]
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[[Category: Edwards, A M.]]
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[[Category: Fedorov O]]
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[[Category: Fedorov, O.]]
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[[Category: Gileadi O]]
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[[Category: Gileadi, O.]]
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[[Category: Krojer T]]
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[[Category: Krojer, T.]]
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[[Category: Mahajan P]]
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[[Category: Mahajan, P.]]
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[[Category: Petrie K]]
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[[Category: Petrie, K.]]
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[[Category: Sanvitale C]]
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[[Category: SGC, Structural Genomics Consortium.]]
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[[Category: Vollmar M]]
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[[Category: Sanvitale, C.]]
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[[Category: Weigelt J]]
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[[Category: Vollmar, M.]]
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[[Category: Von Delft F]]
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[[Category: Weigelt, J.]]
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[[Category: Protein kinase]]
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[[Category: Sgc]]
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[[Category: Structural genomics consortium]]
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[[Category: Transferase]]
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Current revision

Crystal structure of the ACVR1 kinase domain in complex with LDN-193189

PDB ID 3q4u

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