5zok
From Proteopedia
(Difference between revisions)
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| - | '''Unreleased structure''' | ||
| - | The entry | + | ==Crystal structure of human SMAD1-MAN1 complex.== |
| - | + | <StructureSection load='5zok' size='340' side='right' caption='[[5zok]], [[Resolution|resolution]] 2.85Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[5zok]] is a 4 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5ZOK OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5ZOK FirstGlance]. <br> | |
| - | + | </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=5zok FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5zok OCA], [http://pdbe.org/5zok PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5zok RCSB], [http://www.ebi.ac.uk/pdbsum/5zok PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5zok ProSAT]</span></td></tr> | |
| - | [[Category: | + | </table> |
| + | == Disease == | ||
| + | [[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> [[http://www.uniprot.org/uniprot/MAN1_HUMAN MAN1_HUMAN]] Isolated osteopoikilosis;Buschke-Ollendorff syndrome;12q14 microdeletion syndrome;Melorheostosis with osteopoikilosis. The disease is caused by mutations affecting the gene represented in this entry. | ||
| + | == Function == | ||
| + | [[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> [[http://www.uniprot.org/uniprot/MAN1_HUMAN MAN1_HUMAN]] Can function as a specific repressor of TGF-beta, activin, and BMP signaling through its interaction with the R-SMAD proteins. Antagonizes TGF-beta-induced cell proliferation arrest.<ref>PMID:15601644</ref> <ref>PMID:15647271</ref> | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
| + | [[Category: Ito, T]] | ||
| + | [[Category: Miyazono, K]] | ||
| + | [[Category: Tanokura, M]] | ||
| + | [[Category: Complex]] | ||
| + | [[Category: Dna binding protein]] | ||
| + | [[Category: Tgf-beta signaling]] | ||
| + | [[Category: Transcription]] | ||
Revision as of 07:52, 17 October 2018
Crystal structure of human SMAD1-MAN1 complex.
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