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2lb0

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==Structure of the first WW domain of human Smurf1 in complex with a di-phosphorylated human Smad1 derived peptide==
==Structure of the first WW domain of human Smurf1 in complex with a di-phosphorylated human Smad1 derived peptide==
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<StructureSection load='2lb0' size='340' side='right' caption='[[2lb0]], [[NMR_Ensembles_of_Models | 21 NMR models]]' scene=''>
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<StructureSection load='2lb0' size='340' side='right'caption='[[2lb0]]' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[2lb0]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2LB0 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2LB0 FirstGlance]. <br>
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<table><tr><td colspan='2'>[[2lb0]] is a 2 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=2LB0 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2LB0 FirstGlance]. <br>
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</td></tr><tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=SEP:PHOSPHOSERINE'>SEP</scene></td></tr>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=SEP:PHOSPHOSERINE'>SEP</scene></td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2law|2law]], [[2lax|2lax]], [[2lay|2lay]], [[2laz|2laz]], [[2lb1|2lb1]], [[2lb2|2lb2]], [[2lb3|2lb3]]</td></tr>
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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=2lb0 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2lb0 OCA], [https://pdbe.org/2lb0 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2lb0 RCSB], [https://www.ebi.ac.uk/pdbsum/2lb0 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2lb0 ProSAT]</span></td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">SMURF1, KIAA1625 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
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<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=2lb0 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2lb0 OCA], [http://pdbe.org/2lb0 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2lb0 RCSB], [http://www.ebi.ac.uk/pdbsum/2lb0 PDBsum]</span></td></tr>
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</table>
</table>
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== Disease ==
 
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[[http://www.uniprot.org/uniprot/SMAD1_HUMAN SMAD1_HUMAN]] Defects in SMAD1 may be a cause of primary pulmonary hypertension (PPH1) [MIM:[http://omim.org/entry/178600 178600]]. A rare disorder characterized by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. The disease can occur from infancy throughout life and it has a mean age at onset of 36 years. Penetrance is reduced. Although familial PPH1 is rare, cases secondary to known etiologies are more common and include those associated with the appetite-suppressant drugs.<ref>PMID:21898662</ref>
 
== Function ==
== Function ==
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[[http://www.uniprot.org/uniprot/SMUF1_HUMAN SMUF1_HUMAN]] E3 ubiquitin-protein ligase that acts as a negative regulator of BMP signaling pathway. Mediates ubiquitination and degradation of SMAD1 and SMAD5, 2 receptor-regulated SMADs specific for the BMP pathway. Promotes ubiquitination and subsequent proteasomal degradation of TRAF family members and RHOA.<ref>PMID:10458166</ref> <ref>PMID:19937093</ref> <ref>PMID:21402695</ref> [[http://www.uniprot.org/uniprot/SMAD1_HUMAN SMAD1_HUMAN]] Transcriptional modulator activated by BMP (bone morphogenetic proteins) type 1 receptor kinase. SMAD1 is a receptor-regulated SMAD (R-SMAD). SMAD1/OAZ1/PSMB4 complex mediates the degradation of the CREBBP/EP300 repressor SNIP1.<ref>PMID:12097147</ref>
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[https://www.uniprot.org/uniprot/SMUF1_HUMAN SMUF1_HUMAN] E3 ubiquitin-protein ligase that acts as a negative regulator of BMP signaling pathway. Mediates ubiquitination and degradation of SMAD1 and SMAD5, 2 receptor-regulated SMADs specific for the BMP pathway. Promotes ubiquitination and subsequent proteasomal degradation of TRAF family members and RHOA.<ref>PMID:10458166</ref> <ref>PMID:19937093</ref> <ref>PMID:21402695</ref>
<div style="background-color:#fffaf0;">
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
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==See Also==
==See Also==
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*[[Ubiquitin protein ligase|Ubiquitin protein ligase]]
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*[[Ubiquitin protein ligase 3D structures|Ubiquitin protein ligase 3D structures]]
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Human]]
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[[Category: Homo sapiens]]
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[[Category: Aragon, E]]
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[[Category: Large Structures]]
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[[Category: Escobedo, A]]
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[[Category: Aragon E]]
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[[Category: Goerner, N]]
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[[Category: Escobedo A]]
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[[Category: Macias, M J]]
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[[Category: Goerner N]]
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[[Category: Massague, J]]
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[[Category: Macias MJ]]
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[[Category: Xi, Q]]
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[[Category: Massague J]]
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[[Category: Zaromytidou, A]]
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[[Category: Xi Q]]
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[[Category: Cdk]]
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[[Category: Zaromytidou A]]
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[[Category: Signal transduction]]
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[[Category: Signaling protein-transcription complex]]
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[[Category: Smad]]
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[[Category: Smurf]]
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Current revision

Structure of the first WW domain of human Smurf1 in complex with a di-phosphorylated human Smad1 derived peptide

PDB ID 2lb0

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